What is polycythemia vera and is it treatable. Nutrition for erythrocytosis

The disease occurs predominantly in the elderly (average age of onset is approximately 60 years), but is also diagnosed in young people and children. For younger patients, a more severe course of the disease is characteristic. Men are slightly more prone to polycythemia vera than women, but young patients are characterized by an inverse proportion.

Causes and risk factors

The causes contributing to the occurrence of true polycythemia have not been finally established. Pathology can be both hereditary and acquired. Found a family predisposition to the disease. In patients with true polycythemia, gene mutations are detected that are inherited in an autosomal recessive manner.

Risk factors include:

  • impact on the body of toxic substances;
  • ionizing radiation;
  • x-ray exposure;
  • extensive burns;
  • long-term use of a number of drugs (gold salts, etc.);
  • advanced forms of tuberculosis;
  • distress;
  • viral diseases;
  • tumor neoplasms;
  • smoking;
  • endocrine disorders caused by tumors of the adrenal glands;
  • heart defects;
  • diseases of the liver and / or kidneys;
  • extensive surgical interventions.

Forms of the disease

True polycythemia is of two types:

  • primary (not a consequence of other pathologies);
  • secondary (develops against the background of other diseases).

Stages of the disease

There are three stages in the clinical picture of polycythemia vera:

  1. Initial (malosymptomatic) - clinical manifestations are insignificant, duration is about 5 years.
  2. The erythremic (expanded) stage lasting 10–20 years, in turn, is divided into substages: IIA - myeloid metaplasia of the spleen is absent; IIB - the presence of myeloid metaplasia of the spleen;
  3. Stage of posterythremic myeloid metaplasia (anemic) with or without myelofibrosis; capable of developing into chronic or acute leukemia.

Symptoms

Polycythemia vera is characterized by a long asymptomatic course. The clinical picture is associated with an increased production of red blood cells in the bone marrow, which is often accompanied by an increase in the number of other cellular elements in the blood. An increase in platelet count leads to vascular thrombosis, which can cause strokes, myocardial infarction, transient ischemic attacks, etc.

In the later stages of the disease, there may be:

  • skin itching, aggravated by exposure to water;
  • bouts of pressing pain behind the sternum during physical exertion;
  • weakness, increased fatigue;
  • memory disorder;
  • headaches, dizziness;
  • erythrocyanosis;
  • eye redness;
  • visual impairment;
  • increased blood pressure;
  • spontaneous bleeding, ecchymosis, gastrointestinal bleeding;
  • varicose veins (especially neck veins);
  • short-term intense pain in the fingertips;
  • stomach ulcer and / or duodenal ulcer;
  • joint pain;
  • heart failure.

Diagnostics

The diagnosis of polycythemia vera is established on the basis of data obtained during the examination:

  • collection of anamnesis;
  • objective examination;
  • general and biochemical blood test;
  • general urine analysis;
  • trepanobiopsy followed by histological analysis of the biopsy;
  • ultrasound examination;
  • computed or magnetic resonance imaging;
  • molecular genetic analysis.

Diagnostic criteria for polycythemia vera:

  • increased mass of circulating erythrocytes: in men - more than 36 ml / kg, in women - more than 32 ml / kg;
  • leukocytes - 12 × 10 9 / l and more;
  • platelets - 400 × 10 9 / l and more;
  • increase in hemoglobin up to 180–240 g/l;
  • an increase in oxygen saturation in arterial blood - 92% or more;
  • an increase in the serum content of vitamin B 12 - 900 pg / ml or more;
  • increased activity of alkaline phosphatase of leukocytes up to 100;
  • splenomegaly.

Differential diagnosis is required with absolute and relative (false) erythrocytosis, neoplasms, hepatic vein thrombosis.

Treatment

Treatment of polycythemia vera is aimed primarily at preventing the development of leukemia, as well as the prevention and / or therapy of thrombohemorrhagic complications. Symptomatic therapy is carried out in order to improve the patient's quality of life.

To reduce blood viscosity in hyperviscosity syndrome, a course of phlebotomy (exfusion, bloodletting) is performed. However, with initially high thrombocytosis, phlebotomy can contribute to the occurrence of thrombotic complications. Myelosuppressive therapy is indicated for patients who do not tolerate bloodletting, as well as in childhood and adolescence.

Interferon preparations are prescribed for a long course (2-3 months) to reduce myeloproliferation, thrombocythemia, and also to prevent the development of vascular complications.

With the help of hardware methods of therapy (erythrocytapheresis, etc.), excess blood cells are removed. In order to prevent thrombosis, anticoagulants are prescribed. To reduce the manifestations of itching, antihistamines are used. In addition, patients are advised to adhere to a dairy-vegetarian diet and limit physical activity.

With a pronounced increase in the size of the spleen (hypersplenism), splenectomy is indicated for patients.

Possible complications and consequences

Polycythemia vera can be complicated by:

  • myelofibrosis;
  • spleen infarction;
  • anemia;
  • nephrosclerosis;
  • cholelithiasis and / or urolithiasis;
  • gout;
  • myocardial infarction;
  • ischemic stroke;
  • cirrhosis of the liver;
  • pulmonary embolism;
  • acute or chronic leukemia.

Forecast

With timely diagnosis and treatment, survival exceeds 10 years. Without adequate therapy, 50% of patients die within 1-1.5 years from the moment of diagnosis.

Prevention

Due to the fact that the exact causes of the disease are unclear, effective methods for the prevention of polycythemia vera have not yet been developed.

Nutrition for polycythemia vera

Almost all diseases of the hematopoietic system occur with the phenomena of anemia, in which the number of red blood cells and hemoglobin decreases.

Anemia is a decrease in the amount of hemoglobin per unit volume of blood, more often with a simultaneous decrease in the number of red blood cells (or the total volume of red blood cells). The term "anemia" without specification does not define a specific disease, but indicates changes in blood tests, i.e. anemia is one of the symptoms of various pathological conditions.

Anemia can be an independent disease or a consequence of another pathological process.

  1. Anemia due to impaired hemoglobin synthesis and iron metabolism (Hypochromic microcytic).
    1. Iron deficiency anemia.
    2. Thalassemia.
    3. Anemia sideroblastic.
    4. Anemia in chronic diseases (in 60% hypochromic normocytic).
  2. Anemia due to impaired DNA synthesis (Hyperchromic macrocytic with megaloblastic type of hematopoiesis).
    1. Anemia pernicious and other B12 deficiency anemias.
    2. Anemia folic acid deficiency.
  3. Other pathogenetic mechanisms (usually normochromic normocytic anemia).
    1. Aplastic anemia.
    2. hypoplastic anemia.
    3. Myelophthisic anemia.
    4. Acute posthemorrhagic anemia.
    5. hemolytic anemia.

The most common (up to 80% of cases) in clinical practice are anemia caused by iron deficiency. Iron deficiency anemia is a disease caused by the depletion of iron stores in the body, which entails a violation of the synthesis of iron-containing proteins.

Due to a violation of the synthesis of iron-containing proteins, the body is not sufficiently provided with oxygen, respiratory processes in cells, tissues and organs are suppressed with the development of dystrophy in them and a violation of their functions.

The main factor determining the level of plasma iron is the interaction of the processes of synthesis and decay of erythrocytes. For the needs of hematopoiesis, iron is used from the blood depot. Losses of iron are replenished with food iron. The body of an adult contains about 3-5 g of iron in bound form. Read more: The biological role of iron. 70% of the body's iron is found in bound form. The daily requirement for iron in general must be provided by nutrition. Therefore, the nutritional factor is of particular importance in the development of iron deficiency anemia.

The risk group for the development of anemia includes women of childbearing age (due to pregnancy and chronic menstrual blood loss), children due to an increased need for iron, and people who do not consume enough iron-containing foods: vegetarians, the elderly and the elderly.

Only 10–20% of the iron supplied with food is absorbed, and the amount of iron consumed should be 5–10 times the daily requirement. The daily requirement for iron intake is on average 10 mg/day for men and mg/day for women.

If the balance of iron intake in the body is negative, then the iron depot in the body is activated.

Iron losses occur through the intestines, as well as with urine, sweat, epithelium, hair and nails. In a man, iron losses are almost 1 mg / day. Women of childbearing age lose approximately 40–200 mg during menstruation, resulting in an average loss of almost 1.8–2 mg/day.

The task of diet therapy for anemia is to provide the body with the nutrients necessary for hematopoiesis, primarily iron, against the background of physiological nutrition. The role of individual products as sources of iron is determined not so much by their quantity, but by the degree of absorption of iron from them.

  • Basic principles of building a diet for patients with iron deficiency anemia
    • Achieve the efficiency of iron absorption from various foods in the intestine.
    • Balance the ratio of heme and non-heme iron compounds in food, taking into account the iron content in various foods.
    • Balance the amount of intake of substances that enhance and inhibit the absorption of iron.
    • Balance the content of proteins, fats, carbohydrates in food.
    • Choose an adequate caloric intake.

Healthy people absorb approximately 5 to 10% of the iron in their diet, while those who are iron deficient absorb approximately 10–20%. The absorption of iron from food depends on many factors. Heme iron, which is found in animal products, is better absorbed. The absorption of iron from plant foods is enhanced with a mixed diet (foods containing heme iron increase the absorption of non-heme iron).

Adding meat and meat products or fish to each meal increases the absorption of iron from plant-based foods.

The addition of citrus juice, fruits of other fruits and berries without pulp, rosehip broth, compote with the addition of ascorbic acid (25–50 mg) or citric acid also increases the absorption of iron, since ascorbic acid plays a major physiological role in iron absorption. When using fruit juice from citrus fruits, the absorption of iron from cereals, bread, eggs increases, although there is little iron in citrus fruits themselves.

Suppresses the absorption of iron strong tea, as well as a high content of dietary fiber in the diet (wheat bran, for example, maximally prevent the absorption of iron from bread). Oxalic acid and tannins impair iron absorption, so spinach, sorrel, rhubarb, blueberries, dogwood, persimmon, chokeberry or quince, rich in them, are not significant sources of iron. Worse the absorption of iron egg yolk, cocoa, chocolate.

Iron is poorly absorbed from foods high in phytate - wheat germ, bean oil, spinach, lentils, and beet greens.

Ferrous sulphate, iron gluconate, and iron glycerophosphate are often added to foodstuffs. In addition, highly purified reduced iron is used. Milk, grain products, bread, rice, table salt, sugar and fruit juices are enriched with it.

Vitamin B12 deficiency anemia is a disease caused by the depletion of vitamin B12 (cyanocobalamin) reserves in the body, which leads to a violation of DNA synthesis in cells. The disease is manifested by a sharp violation of hematopoiesis with the development of hyperchromic macrocytic anemia with a megaloblastic type of hematopoiesis, neutropenia, thrombocytopenia, and dysfunction of the nervous system.

The main food sources of vitamin B12 include organ meats, sea fish, and dairy products.

Nutrition in the absence of concomitant diseases of the gastrointestinal tract can be based on diet number 11. Currently, hospitals recommend using a higher protein diet option (high protein diet).

  • Nutrition in the prevention and treatment of folic acid deficiency anemia

Folate deficiency anemia is a disease caused by the depletion of folic acid reserves in the body, which leads to a violation of DNA synthesis in cells. The disease is manifested by the development of hyperchromic macrocytic anemia.

The absorption of folacin is reduced in alcoholics under the influence of ethanol, while taking certain drugs (pentamidine, triamterene, trimethoprim, phenytoin, methotrexate, aminopterin, barbiturates, sulfonamides), as well as in an acidic environment. An increased need for folacin is present in patients with enteritis, hemolytic anemia, skin diseases, during pregnancy and lactation. Folacin deficiency during pregnancy can lead to impaired mental development and deformities in children.

It is believed that the diet can provide approximately 2/3 of the daily requirement for folacin, and 1/3 is synthesized by the intestinal microflora. The vitamin is significantly destroyed during heat treatment (by 80–90%) and during grinding of products.

Nutrition in the absence of concomitant diseases of the gastrointestinal tract can be based on diet number 11. Currently, hospitals recommend using a higher protein diet option (high protein diet).

When preparing special diets for patients with leukopenia and thrombocytopenia, it is advisable to include in the diet products containing substances necessary for building the stroma of blood elements, hemoglobin synthesis, differentiation and maturation of blood cells. It is also important to exclude substances that have an inhibitory effect on certain aspects of hematopoiesis.

The composition of the diet for leukopenia and agranulocytosis corresponds to that for anemia, with changes towards an increase in folic and ascorbic acids, vitamin B12 in food; complete protein (amino acids - methionine, choline, lysine).

It is believed that with leukopenia and agranulocytosis, purine metabolism sharply increases, therefore, the amount of meat, liver, kidneys is reduced in the diet, but the amount of protein due to vegetable (soy) increases. Limit animal fat and increase the amount of vegetable oil. Preference is given to vegetables, fruits, berries, herbs.

Principles of therapeutic nutrition for leukopenia and thrombocytopenia:

  • To build the stroma of blood cells, a sufficient amount of proteins containing amino acids such as lysine, methionine, tryptophan, tyrosine, lecithin, and choline should be administered.
  • Cobalt, vitamin B 12, folic and ascorbic acids are necessary for the differentiation of blood elements, as well as for the transformation of yellow bone marrow into active red. In addition, sufficient intake of vitamin B 6 (pyridoxine) and riboflavin is necessary.
  • To stimulate and regulate the release of ready-made blood cells from the blood depots, thiamine and nitrogenous extractives are needed.
  • In the diet for patients with reduced hematopoiesis moderately restrict the introduction of fat, foods rich in lead, aluminum, selenium and gold. These substances have a negative effect on some phases of erythropoiesis and leukopoiesis.

Nutrition in the absence of concomitant diseases of the gastrointestinal tract can be based on diet number 11. Currently, hospitals recommend using a higher protein diet option (high protein diet).

The pathogenetic principles of diet therapy for acute leukemia are based on improving the viability of the immune system, correcting metabolic disorders, reducing the manifestations of intoxication, and correcting vitamin deficiency. A physiologically complete and easily digestible diet is needed, enriched with vitamin C and B vitamins, as well as sources of iron and other hematopoietic trace elements: cobalt, copper, manganese, nickel, zinc, molybdenum. Arsenic has a cytostatic effect. Vanadium, titanium, chromium affect redox processes.

Nutrition in the absence of concomitant diseases of the gastrointestinal tract can be based on diet number 11. Currently, hospitals recommend using a higher protein diet option (high protein diet).

Special requirements for diet therapy for certain types of chemotherapy:

  • In the treatment of glucocorticosteroids, there is an increase in the breakdown of protein in the body, the formation of fat in it increases, the body's tolerance to glucose decreases, sodium and water are retained, the excretion of potassium and calcium increases, irritation of the mucous membranes is observed. Accordingly, it is necessary to increase the protein quota in the diet to 120 g / day, while 60–65% is due to animal protein, low-fat cottage cheese, fish, seafood, lean meat, egg white. Reduce the amount of carbohydrates to 300 g, and primarily at the expense of simple carbohydrates (sugar and products containing it). Recommended dishes from oatmeal, buckwheat. In the diet, it is necessary to limit fat to 70–75 g, of which 30–35% should be vegetable fats. Reduce the content of table salt (up to 4 g / day), oxalic acid, cholesterol. Increase the content of potassium, calcium, vitamins C, A, D, E. Free fluid intake is limited. Culinary processing follows the principles of mechanical, thermal and chemical sparing.
  • In the treatment of cytostatics, particular importance is attached to reducing intoxication and sparing the digestive tract.
  • Therapeutic nutrition for erythremia

    In the presence of erythremia, the hematopoietic activity of the bone marrow increases, which leads to an increase in blood mass due to erythrocytes, and sometimes also leukocytes and platelets - polycythemia.

    In the initial stage of the disease, physiologically nutritious nutrition is recommended - diet No. 15 is recommended. Limit foods that enhance blood formation, such as the liver. Recommend food rich in fat (up to 150-200 g / day), dairy, vegetable products.

    In the advanced stage of erythremia, a diet similar to the diet for gout is shown, in which meat and fish products, especially meat of internal organs, legumes, are sharply limited or excluded - diet No. 6 is recommended.

Nutrition for erythremia, general principles

Erythremia is, in simple terms, a thickening of the blood when free erythrocytes (red blood cells) increase, and with them hemoglobin. This condition threatens the development of thrombosis, therefore, it requires an integrated approach to treatment, including dietary nutrition.

The main principle and goal of therapeutic nutrition in this disease is to thin the blood by drinking large amounts of purified water and reducing certain protein products that enhance the production of red blood cells.

The treatment table according to Pevzner No. 6 meets these requirements as much as possible for a long time, until the blood counts are normalized.

Features of table number 6

Diet food of the sixth table, which is used in the treatment of erythremia, prohibits the use of foods such as:

  • Fruits and vegetables of red colors, as well as dishes and drinks from them (tomatoes, pomegranates, beets, red varieties of apples, etc.)
  • Meat and dishes from it, especially beef and liver, should be reduced to no more than 1-2 times a week
  • Products with a large number of dyes and preservatives (shop canned food, sausages, smoked meats, sauces, as well as fast food) - contribute to increased thrombosis
  • Alcohol of any origin - reduce to a minimum or completely stop (no more than 30 g of spirits or a glass of white wine per month) - destroys spleen and liver cells
  • Products with vitamin C (citrus fruits, apples, etc.) - enhances the accumulation of iron in the body and contributes to an even greater increase in hemoglobin levels in the blood

Among the useful and permitted products during the period of exacerbation of erythremia, the following are distinguished:

  • Foods based on whole grains of various cultures - whole grain bread, brown rice, bran, etc.
  • Eggs are an ideal source of essential proteins and fats in the body.
  • Dairy products (milk, sour cream, fermented baked milk, yogurt, kefir, etc.) - without dyes, preferably homemade
  • Vegetables in any form, with the exception of red ones (white beans are especially effective)
  • Greens (parsley, spinach, dill, sorrel, lettuce)
  • Dried apricots and raisins in small quantities
  • Nuts, especially brazil nuts and almonds
  • Green tea and herbal tinctures

The frequency of food intake with such a diet is 4-6 times a day, in relatively small portions. Between meals, on an empty stomach, be sure to drink plain purified water in unlimited quantities, but not less than 2 liters per day (including edematous conditions).

More about protein foods for erythremia

Despite the fact that protein foods are the main food in the diet of every person, in the case of erythroemia, this component must be corrected one of the first. Emphasis should be placed on milk proteins and eggs, they have a beneficial effect on hematopoiesis and blood flow, and do not allow excessive production of blood cells.

All meat products, including fish dishes, should be removed from the diet of such patients as much as possible. This is especially true for the liver, kidneys and other internal organs, as well as any part of beef. This also applies to vegetable proteins (their maximum amount is in legumes), with the exception of white beans. These products increase the production of red blood cells and hemoglobin, aggravating the clinical picture of the disease. The frequency of taking meals from these products should not be more than once a week.

More about fatty foods for erythremia

Fats are quite an important component of food, providing a person with a stable supply of energy, as well as other vital functions and mechanisms. However, despite this, all fats are quite heavy food, they overload the patient's body with thickened blood and contribute to the deterioration of the condition and laboratory parameters. These foods include vegetable oils, butter, meat and milk fat, and eggs.

To ensure the maximum recovery process in the blood-forming organs, the daily intake of fats should not exceed 70 g. This is ensured by the almost complete rejection of meat and fish, the intake of low-fat dairy products, and the reduction in the use of oils in cooking.

More about carbohydrates for erythremia

All carbohydrates are a good source of energy for humans, and some of them also contain fiber, which is so useful for digestion. However, you should clearly distinguish between light and heavy carbohydrates. The first group includes simple sugars: potatoes, wheat (peeled grains), white polished rice and regular sugar. These products give a huge release of energy in a short time and, if not consumed, are transformed into fats (subcutaneous and around the internal organs), they should be consumed only before heavy physical exertion. The second group provides a gradual release of energy, is not able to pass into internal fat and ensures the normal functioning of the body. These are products such as cereals (buckwheat, brown rice, red and black rice, pearl barley, millet, barley porridge, etc.), whole grain flour and products from it, fruits and vegetables, honey, cane sugar.

In the case of erythremia, the amount of light (fast) carbohydrates should be minimized, because additional internal fat gives complications to the vascular system (increases the risk of thrombosis, embolism and disrupts blood composition). You can use them before going to the gym or before active sexual intercourse. In all other cases, eat slow carbohydrates.

More about water for erythremia

Water is one of the important aspects of the diet when red blood cells are elevated in blood tests. Due to its penetrating ability, it easily enters the systemic circulation and is able to thin the blood. It should be drunk at least 2-2.5 liters per day, in small sips, between meals (on an empty and empty stomach). The duration of such forced and volumetric fluid intake depends on the individual characteristics of the patient and continues until complete normalization in the general blood test. During the period of remission, the amount of water drunk per day should be at least 1.5 liters per day.

The article was prepared by nutritionist Azarova Marina Andreevna

Polycythemia (erythremia, Wakez's disease): causes, signs, course, therapy, prognosis

Polycythemia is a disease that can be assumed just by looking at the patient's face. And if you still conduct the necessary blood test, then there will be no doubt at all. In reference books, it can also be found under other names: erythremia and Wakez's disease.

Redness of the face is quite common and there is always an explanation for this. In addition, it is short-term and does not linger for a long time. Various reasons can cause sudden reddening of the face: fever, high blood pressure, hot flashes during menopause, a recent tan, an awkward situation, and emotionally labile people tend to blush often, even if others do not see any prerequisites for this.

Polycythemia is different. Here, the redness is persistent, not transient, evenly distributed over the entire face. The color of an overly “healthy” plethora is saturated, bright cherry.

What kind of disease is polycythemia?

True polycythemia (erythremia, Wakez's disease) belongs to the group of hemoblastoses (erythrocytosis) or chronic leukemia with a benign course. The disease is characterized by the growth of all three hematopoiesis with a significant advantage of erythrocyte and megakaryocytic, due to which there is an increase not only in the number of red blood cells - erythrocytes, but also other blood cells that originate from these germs, where the source of the tumor process is the affected precursor cells of myelopoiesis. It is they who begin uncontrolled proliferation and differentiation into mature forms of erythrocytes.

Most affected under such conditions are immature red blood cells, which are hypersensitive to erythropoietin even at low doses. With polycythemia, the growth of leukocytes of the granulocytic series (primarily stab and neutrophils) and platelets. The cells of the lymphoid series, which include lymphocytes, are not affected by the pathological process, since they come from a different germ and have a different way of reproduction and maturation.

Cancer or not cancer?

Erythremia - not to say that it occurs all the time, however, there are a couple of people in a town of 25 thousand people, while for some reason men of 60 or so “love” this disease more, although any person can meet with such a pathology age. True, for newborns and young children, true polycythemia is absolutely not typical, so if erythremia is found in a child, then most likely she will wear secondary character and be a symptom and consequence of another disease (toxic dyspepsia, stress erythrocytosis).

For many people, a disease classified as leukemia (whether acute or chronic) is primarily associated with blood cancer. Here it is interesting to understand: is it cancer or not? In this case, it would be more expedient, clearer and more correct to talk about the malignancy or benignity of true polycythemia in order to determine the boundary between "good" and "evil". But, since the word "cancer" refers to tumors from epithelial tissues, then in this case this term is inappropriate, because this tumor comes from hematopoietic tissue.

Wakez disease refers to malignant tumors, but it is characterized by high differentiation of cells. The course of the disease is long and chronic, for the time being qualified as benign. However, such a course can only last up to a certain point, and then with proper and timely treatment, but after a certain period of time, when significant changes in erythropoiesis occur, the disease becomes acute and acquires more “evil” features and manifestations. Here it is - true polycythemia, the prognosis of which will completely depend on how quickly its progression occurs.

Why do sprouts grow incorrectly?

Any patient suffering from erythremia, sooner or later asks the question: "Why did this" illness happen "to me?". The search for the cause of many pathological conditions is usually useful and gives certain results, increases the effectiveness of treatment and promotes recovery. But not in the case of polycythemia.

The causes of the disease can only be assumed, but not definitely stated. Only one clue can be for the doctor when determining the origin of the disease - genetic abnormalities. However, the pathological gene has not yet been found, so the exact localization of the defect has not yet been determined. There are, however, suggestions that Wakez's disease may be associated with trisomy 8 and 9 pairs (47 chromosomes) or other violation of the chromosomal apparatus, for example, the loss of a segment (deletion) of the long arm C5, C20, but this is still guesswork, although built on conclusions of scientific research.

Complaints and clinical picture

If there is nothing to say about the causes of polycythemia, then we can talk about clinical manifestations for a long time and a lot. They are bright and varied, since already from the 2nd degree of development of the disease, literally all organs are drawn into the process. Subjective sensations of the patient are of a general nature:

  • Weakness and constant feeling of fatigue;
  • Significant decrease in performance;
  • increased sweating;
  • Headaches and dizziness;
  • Marked memory loss;
  • Visual and auditory disorders (decrease).

Complaints characteristic of this disease and characterized by it:

  • Acute burning pain in the fingers and toes (the vessels are clogged with platelets and red blood cells, which form small aggregates there);
  • Soreness, though not so burning, in the upper and lower limbs;
  • Itching of the body (a consequence of thrombosis), the intensity of which increases markedly after a shower and a hot bath;
  • Periodic appearance of a rash such as urticaria.

It's obvious that cause all these complaints microcirculation disorder.

reddening of the skin with polycythemia

As the disease progresses, more and more new symptoms are formed:

  1. Hyperemia of the skin and mucous membranes due to the expansion of capillaries;
  2. Pain in the region of the heart, resembling angina pectoris;
  3. Pain in the left hypochondrium caused by overload and enlargement of the spleen due to the accumulation and destruction of platelets and red blood cells (it is a kind of depot for these cells);
  4. Enlargement of the liver and spleen;
  5. Peptic ulcer of the stomach and 12 duodenal ulcer;
  6. Dysuria (difficulty urinating) and pain in the lumbar region due to the development of uric acid diathesis, which was caused by a shift in blood buffer systems;
  7. Pain in bones and joints as a result hyperplasia(overgrowth) bone marrow;
  8. Gout;
  9. Manifestations of hemorrhagic nature: bleeding (nasal, gingival, intestinal) and skin hemorrhages;
  10. Injections of the vessels of the conjunctiva, which is why the eyes of such patients are called "rabbit eyes";
  11. Telangiectasias;
  12. Tendency to thrombosis of veins and arteries;
  13. Varicose veins of the lower leg;
  14. Thrombophlebitis;
  15. Possible thrombosis of the coronary vessels with the development of myocardial infarction;
  16. Intermittent claudication, which may result in gangrene;
  17. Arterial hypertension (almost 50% of patients), giving rise to a tendency to strokes and heart attacks;
  18. Respiratory damage due to immune disorders, which cannot adequately respond to infectious agents that cause inflammation. In this case, red blood cells begin to behave like suppressors and suppress the immunological response to viruses and tumors. In addition, they are in the blood in an abnormally high amount, which further aggravates the state of the immune system;
  19. The kidneys and urinary tract suffer, so patients have a tendency to pyelonephritis, urolithiasis;
  20. The central nervous system does not remain aloof from the events taking place in the body, when it is involved in the pathological process, symptoms of cerebrovascular accident, ischemic stroke (with thrombosis), hemorrhage (less often), insomnia, memory impairment, mnestic disorders appear.

Asymptomatic to end stage

Due to the fact that polycythemia in the early stages is characterized by an asymptomatic course, the above manifestations do not occur in one day, but accumulate gradually and for a long time, it is customary to distinguish 3 stages in the development of the disease.

Initial stage. The patient's condition is satisfactory, the symptoms are moderately severe, the duration of the stage is about 5 years.

Stage of developed clinical manifestations. It takes place in two stages:

II A - proceeds without myeloid metaplasia of the spleen, subjective and objective symptoms of erythremia are present, the duration of the period is years;

II B - myeloid metaplasia of the spleen appears. This stage is characterized by a clear picture of the disease, the symptoms are pronounced, the liver and spleen are significantly enlarged.

The terminal stage, which has all the signs of a malignant process. Complaints of the patient are diverse, "everything hurts, everything is wrong." At this stage, cells lose their ability to differentiate, which creates a substrate for leukemia, which replaces chronic erythremia, or rather, it turns into acute leukemia.

The terminal stage is characterized by a particularly severe course (hemorrhagic syndrome, rupture of the spleen, infectious and inflammatory processes that cannot be treated due to deep immunodeficiency). It usually ends in death soon after.

Thus, the life expectancy for polycythemia is years, which may be not bad, especially considering that the disease can overtake after 60. And this means that there is a certain prospect of living up to 80 years. However, the prognosis of the disease still depends most of all on its outcome, that is, on what form of leukemia erythremia is transformed into at stage III (chronic myeloid leukemia, myelofibrosis, acute leukemia).

Diagnosis of Wakez disease

The diagnosis of polycythemia vera is mainly based on laboratory data with the determination of the following indicators:

  • Complete blood count, in which you can notice a significant increase in red blood cells (6.0-12.0 x / l), hemoglobin (g / l), hematocrit (ratio of plasma and red blood). The number of platelets can reach levels of 10 9 /l, while they can significantly increase in size, and leukocytes - up to 9.0-15.0 x 10 9 /l (due to rods and neutrophils). ESR with true polycythemia is always reduced and can reach zero.

Morphologically, erythrocytes do not always change and often remain normal, but in some cases, erythremia can be observed anisocytosis(erythrocytes of different sizes). Platelets indicate the severity and prognosis of the disease with polycythemia in the general blood test (the more there are, the more severe the course of the disease);

  • BAC (biochemical blood test) with the determination of the level of alkaline phosphatase and uric acid. For erythremia, the accumulation of the latter is very characteristic, which indicates the development of gout (a consequence of Wakez's disease);
  • Radiological examination using radioactive chromium helps to determine the increase in circulating red blood cells;
  • Sternal puncture (bone marrow sampling from the sternum) followed by cytological diagnosis. In the preparation hyperplasia of all three germs with a significant predominance of red and megakaryocytic;
  • Trepanobiopsy(histological examination of material taken from the ilium) is the most informative method that allows you to most reliably identify the main symptom of the disease - three-growth hyperplasia.

In addition to hematological parameters, to establish the diagnosis of polycythemia vera, the patient is sent for an ultrasound examination (ultrasound) of the abdominal organs (enlargement of the liver and spleen).

So, the diagnosis is established ... What's next?

And then the patient is waiting for treatment in the hematological department, where tactics are determined by clinical manifestations, hematological parameters and the stage of the disease. Therapeutic measures for erythremia usually include:

  1. Bloodletting, which allows to reduce the number of red blood cells to 4.5-5.0 x / l and Hb (hemoglobin) to 150 g / l. To do this, with an interval of 1-2 days, 500 ml of blood is taken until the number of erythrocytes and Hb drops. The bloodletting procedure is sometimes replaced by hematologists with erythrocytopheresis, when, after sampling by centrifugation or separation, red blood is separated, and the plasma is returned to the patient;
  2. Cytostatic therapy (myelosan, imifos, hydroxyurea, hydroxycarbamide);
  3. Antiplatelet agents (aspirin, dipyridamole), which, however, require caution in their use. So, acetylsalicylic acid can enhance the manifestation of hemorrhagic syndrome and cause internal bleeding if the patient has a stomach ulcer or duodenal ulcer;
  4. Interferon-α2b, which is successfully used with cytostatics and increases their effectiveness.

The treatment regimen for erythremia is signed by the doctor for each case individually, so our task is only to briefly acquaint the reader with the drugs used to treat Wakez's disease.

Nutrition, diet and folk remedies

A significant role in the treatment of polycythemia is assigned to the regime of work (reduction of physical activity), rest and nutrition. In the initial stage of the disease, when the symptoms are not yet expressed or are weakly manifested, the patient is assigned table number 15 (general), however, with some reservations. The patient is not recommended to consume foods that enhance hematopoiesis (liver, for example) and are offered to revise the diet, giving preference to dairy and vegetable products.

In the second stage of the disease, the patient is assigned table number 6, which corresponds to the gout diet and limits or completely excludes fish and meat dishes, legumes and sorrel. Having been discharged from the hospital, the patient must adhere to the recommendations given by the doctor during outpatient observation or treatment.

Question: "Is it possible to treat folk remedies?" sounds with the same frequency for all diseases. Erythremia is no exception. However, as already noted, the course of the disease and the life expectancy of the patient depend entirely on timely treatment, the purpose of which is to achieve a long and stable remission and delay the third stage for the longest possible time.

During the calm period of the pathological process, the patient must still remember that the disease can return at any time, therefore, he must discuss his life without exacerbation with the attending physician, in whom he is observed, periodically take tests and undergo an examination.

Treatment of blood diseases with folk remedies should not be generalized, and if there are many recipes to increase hemoglobin levels or to thin the blood, this does not mean at all that they are suitable for the treatment of polycythemia, for which, in general, medicinal herbs have not yet been found. Wakez's disease is a delicate matter, and in order to control the function of the bone marrow and thus affect the hematopoietic system, you need to have objective data that can be assessed by a person with certain knowledge, that is, the attending physician.

In conclusion, I would like to say a few words to readers about relative erythremia, which cannot be confused with true erythremia, since relative erythrocytosis can occur against the background of many somatic diseases and successfully end when the disease is cured. In addition, erythrocytosis as a symptom can accompany prolonged vomiting, diarrhea, burn disease and hyperhidrosis. In such cases, erythrocytosis is a temporary phenomenon and is associated primarily with dehydration of the body, when the amount of circulating plasma, which consists of 90% water, decreases.

Prognosis for polycythemia vera

Among blood diseases, there are many that cause a decrease in various elements - erythrocytes, leukocytes, platelets. But in some pathologies, on the contrary, there is an uncontrolled increase in the number of blood cells. The condition in which there is a chronic increase in the number of red blood cells, and other pathological changes occur, is called "true polycythemia".

Features of the disease

Primary (true) polycythemia is a blood disease from the leukemia group that occurs idiopathically (for no apparent reason), proceeds for a long time (chronically) and is characterized by an increase in the number of red blood cells, an increase in hematocrit and blood viscosity. Synonyms for the name of the pathology are Wakez-Osler disease, erythremia, primary erythrocytosis. The consequences of erythrocytosis and blood clotting in this myeloproliferative disease can be serious and relate to the risk of thrombosis, enlargement and malfunction of the spleen, an increase in circulating blood volume, etc.

Erythremia is considered a malignant tumor process, which is caused by increased proliferation (hyperplasia) of bone marrow cells. Especially strongly the pathological process covers the erythroblastic germ - a part of the bone marrow, consisting of erythroblasts and normoblasts. The pathogenesis of the main manifestations is associated with the appearance in the blood of a huge number of red blood cells, as well as with a certain increase in the number of platelets and neutrophils (neutrophilic leukocytes). The blood cells are morphologically normal, but their number is abnormal. As a result, blood viscosity and the amount of blood in the circulating bloodstream increase. The result is a slower blood flow, the formation of blood clots, a violation of the local blood supply to tissues and their hypoxia.

If initially the patient most often has primary erythrocytosis, that is, only the number of erythrocytes increases, then further changes begin to cover other blood cells. Extramedullary hematopoiesis (pathological formation of blood outside the bone marrow) occurs in the organs of the peritoneum - in the liver and spleen, where part of erythropoiesis is also localized - the process of formation of red blood cells. At a late stage of the disease, the life cycle of erythrocytes is shortened, anemia, thrombocytopenia, myelofibrosis may develop, and the precursor cells of leukocytes and erythrocytes enter the general circulation without maturing. In about 10% of cases, the pathology flows into acute leukemia.

The study and first description of erythrocytosis was made in 1892 by Vakez, and in 1903 the scientist Osler suggested that the cause of the disease is a violation of the bone marrow. True polycythemia is observed somewhat more often than other similar pathologies, but still it is quite rare. It is diagnosed in approximately 5 people per year per 1 million population. Most often, the disease occurs in people older than 50 years, the average age of detection is 60 years. In children, a similar diagnosis is made very rarely, mainly after 12 years. On average, only 5% of patients are under 40 years of age. Men suffer from this pathology more often than women. In the general structure of chronic myeloproliferative diseases, polycythemia vera takes the 4th place. Sometimes it is inherited, so there are family cases.

Causes of pathology

The primary form of the disease is considered hereditary, transmitted in an autosomal recessive manner. In this case, it is often referred to as "family polycythemia". But most often, erythremia is a secondary condition, representing one of the manifestations of a general pathological process. The exact causes have not been established, but there are several theories about the appearance of polycythemia vera. Thus, there is a connection between the development of the disease and the transformation of stem cells, when a tyrosine kinase mutation occurs, which occurs in polycythemia vera more often than in other blood diseases.

Studies of cells in erythremia revealed in many patients the clonal origin of the pathology, since the same enzyme was detected in leukocytes, platelets, erythrocytes. The clonal theory is also confirmed by ongoing cytological studies in relation to the karyotype of chromosome groups, where various defects were identified that are similar in different patients. There is also a virus-genetic theory, according to which up to 15 types of viruses can enter the body and, with the participation of a number of provoking factors, lead to a malfunction in the bone marrow. They penetrate into the precursors of blood cells, which then, instead of normal maturation, begin to divide and form new red blood cells and other cells.

As for the risk factors for the development of true polycythemia, then, presumably, they may be as follows:

  • lung diseases;
  • long stay at high altitude above sea level;
  • hypoventilation syndromes;
  • various hemoglobinopathies;
  • long smoking history;
  • tumors of the bone marrow, blood;
  • hemoconcentration with long-term use of diuretics;
  • burns of a large part of the body;
  • severe stress;
  • diarrhea;
  • exposure to x-rays, radiation;
  • poisoning with vapors of chemicals, their ingress through the skin;
  • the intake of toxic substances in the digestive tract;
  • treatment with gold salts;
  • advanced tuberculosis;
  • major surgical interventions;
  • "blue" heart defects;
  • kidney pathology - hydronephrosis, stenosis of the arteries of the kidneys.

Thus, the main cause of secondary erythrocytosis are all conditions that somehow provoke tissue hypoxia, stress for the body or its intoxication. In addition, oncological processes, endocrine pathologies, and liver diseases can have a great impact on the brain and the production of additional blood cells.

Classification of true polycythemia

The disease is classified into the following stages:

  1. The first or initial stage. It can last more than 5 years, is the development of plethoric syndrome, that is, increased blood supply to organs. At this stage, the symptoms can be expressed moderately, complications do not occur. A complete blood count reflects a slight increase in the number of erythrocytes, a bone marrow puncture shows an increase in erythropoiesis or the production of all the main elements of the blood, with the exception of lymphocytes.
  2. The second is the A stage, or polycythemic stage. Duration - from 5 to 15 years. The plethoric syndrome is more pronounced, there is an increase in the spleen, liver (hematopoietic organs), thrombosis is often recorded in the veins and arteries. Tumor growth in the organs of the peritoneum is not observed. If this stage ends with a decrease in the number of platelets - thrombocytopenia, then the patient may experience various bleeding. Frequent hemorrhages cause a lack of iron in the body. A general blood test reflects an increase in erythrocytes, platelets, leukocytes, with a running course - a decrease in platelets. In the myelogram, there is an increased formation of most blood cells (with the exception of lymphocytes), cicatricial changes in the brain are formed.
  3. The second - stage B, or polycythemic stage with myeloid metaplasia of the organ - the spleen. The patient continues to increase the size of the spleen, and often the liver. Puncture of the spleen reveals tumor growth. There are frequent thromboses interspersed with bleeding. In the general analysis, there is an even greater increase in the number of erythrocytes, leukocytes, there are erythrocytes of different sizes, shapes, there are immature precursors of all blood cells. In the bone marrow, the number of cicatricial changes increases.
  4. The third, or anemic stage. It is the outcome of a disease in which the activity of blood cells is depleted. The number of erythrocytes, leukocytes, and platelets is greatly reduced, the liver and spleen are enlarged with myeloid metaplasia, extensive cicatricial changes occur in the bone marrow. A person becomes disabled, most often due to the consequences of thrombosis or the addition of acute leukemia, myelofibrosis, hematopoietic hypoplasia or chronic myeloid leukemia. This stage is recorded approximately one year after the development of the pathology.

Symptoms of manifestation

Often this pathology is asymptomatic, but only in its initial stages. Later, the disease in a patient manifests itself in one way or another, while specific symptoms can be varied. Basically, the symptom complex includes the following main signs:

  1. Change in skin tone, dilated veins. Most often, in the neck of an adult, the veins begin to shine through strongly, their pattern becomes stronger due to swelling, overfilling with blood. But the skin signs become the most obvious: the skin color becomes dark red, literally cherry. Most of all, this is noticeable in the neck, arms, face, which is associated with overfilling of the subcutaneous arteries with blood. At the same time, many patients mistakenly think that blood pressure rises against the background of hypertension, and therefore they often continue to take drugs for pressure and do not go to the doctor. With a careful attitude to health, you can see that the lips and tongue also changed their shade, became red-blue. The vessels of the eyes are also filled with blood, their plethora leads to hyperemia of the sclera and conjunctiva of the organs of vision. The hard palate remains the same color, but the soft palate also becomes brighter, burgundy.
  2. Skin itching. All described changes in the skin in about half of the cases are complemented by severe discomfort and itching. This symptom is very characteristic of erythremia, both primary and secondary. Since after taking water procedures in patients, histamine is released, as well as prostaglandins, itching of the skin may become even more pronounced after a bath or shower.
  3. Pain in limbs. Many people develop obliterating endarteritis, which results in persistent and severe pain in the legs. They can intensify with exercise, long walking, in the evening, at first they are often perceived as a symptom of fatigue in an elderly person. Pain is also observed during palpation and tapping of flat bones, which reflects the process of hyperplasia and scarring in the bone marrow. The next type of pain in a person with polycythemia vera is persistent burning pain in the large and small joints of the legs, which resemble gouty pains and are caused by the same reason as gout - an increase in the level of uric acid. Another type of pain is severe, poorly tolerated pain in the fingers and toes, in which the skin becomes bluish-red, blue spots appear on it. These pains are caused by an increase in the number of platelets and the appearance of microthrombosis of capillaries.
  4. Splenomegaly. The growth of the spleen in size is observed in almost every person with polycythemia vera, but at different stages of the disease. This is due to increased filling of the spleen with blood and the development of myeloproliferative phenomena. Somewhat less often, but still there is a strong increase in the size of the liver - hepatomegaly.
  5. Ulcer disease. Approximately one in ten people with Wackez-Osler disease develop ulcers in the small intestine (often in the duodenum) and in the stomach. This is due to the activation of Helicobacter pylori bacteria, as well as the development of microthrombosis in the gastrointestinal tract.
  6. thrombosis and bleeding. Almost all patients at a certain stage have a tendency to thrombosis, and even until recently, patients died from such complications at an early stage of the disease. Now ongoing modern treatment can prevent the appearance of blood clots in the brain, spleen, legs, which threaten embolism and death. Increased blood viscosity characterizes true polycythemia in the initial stages, and later, against the background of depletion of the platelet formation system, bleeding develops - it is observed in the gums, nose, uterus, and gastrointestinal tract.

There are other signs of polycythemia vera that a person may complain about, but they are not specific and may be inherent in various pathologies:

  • fatigue;
  • head goals;
  • tinnitus;
  • nausea;
  • dizziness;
  • feeling of pulsation in the temples, ears;
  • decreased appetite, performance;
  • the appearance of "flies" before the eyes;
  • other visual impairments - loss of fields, loss of visual acuity;
  • shortness of breath, coughing;
  • increased blood pressure;
  • unexplained weight loss;
  • prolonged subfebrile condition;
  • insomnia;
  • numbness, tingling of the fingers;
  • epileptiform seizures and paralysis (rare).

In general, the disease is characterized by a long and sometimes benign course, especially when adequate treatment is carried out. But in some people, especially those who are not receiving therapy, various consequences of polycythemia vera may appear early.

Possible complications

Most often, complications are associated with thrombosis and embolism of the veins and vessels of the spleen, liver, legs, brain, and other areas of the body. This leads to different consequences depending on the size of the thrombus, the affected area. Transient ischemic attacks, strokes, thrombophlebitis and phlebothrombosis of superficial and deep veins, blockage of retinal blood vessels and blindness, heart attacks of internal organs, myocardial infarction may occur.

In the most advanced stages of the pathology, kidney stones (urolithiasis), gout, nephrosclerosis, and cirrhosis of the liver often appear. Complications are likely due to tissue bleeding - bleeding from gastrointestinal ulcers, anemia. From the side of the heart, in addition to myocardial infarction, signs of myocardiosclerosis and heart failure are also possible. There is also the possibility of the transition of true polycythemia to acute leukemia, chronic leukemia and other oncopathologies.

Carrying out diagnostics

It is not easy to make a diagnosis of this disease, especially in the absence of a characteristic clinical picture and in the presence of only general symptoms. Nevertheless, the totality of data from hematological and biochemical analyzes, as well as some distinctive features of the patient's appearance, coupled with his complaints, will help the doctor determine the cause of the changes.

The main indicators for establishing the diagnosis of polycythemia vera are the indicators of the general blood test - the number of erythrocytes and hematocrit. In men, the development of this disease can be suspected if the number of red blood cells is more than 5.7 * 10 * 9 / l, hemoglobin is more than 177 g / l, hematocrit was above 52%. In women, an excess of indicators is noted if they are more than 5.2 * 10 * 9 / l, 172 g / l, 48-50%, respectively. These figures are typical for the early stages of pathology, and as it develops, they become even higher. In addition, it is important to assess the mass of circulating erythrocytes, which is normally up to 36 ml/kg for men and up to 32 ml/kg for women.

Other blood parameters (according to biochemistry, general analysis and other tests), which, in combination with the described disorders and in combination with each other, reflect the picture of the development of primary or secondary erythrocytosis:

  1. Moderate or severe thrombocytosis (above 400 * 10 * 9 l), neutrophilic leukocytosis (above 12 * 10 * 9 l) with the presence of an increased number of basophils and eosinophils.
  2. Increase in the number of reticulocytes.
  3. The appearance in the blood of myelocytes, metamyelocytes.
  4. Increase in blood viscosity by %.
  5. A strong decrease in ESR.
  6. Increase in the mass of circulating red blood cells.
  7. Increase in alkaline phosphatase, vitamin B12 in serum.
  8. An increase in the amount of uric acid in the serum.
  9. The saturation of blood in the arteries with oxygen is above 92%.
  10. The appearance of erythrocyte colonies in a test tube.
  11. Decrease in erythropoietin.
  12. Change in color index less than 1.

At the stage of myelofibrosis, hemoglobin and erythrocyte indicators can return to normal, but the number of leukocytes increases greatly, their immature forms appear, and the presence of erythroblasts is diagnosed. As for the myelogram, which is obtained by puncturing the bone marrow, the following changes are revealed in it:

  • reduction in the presence of fatty inclusions;
  • increase in erythroblasts, normoblasts;
  • hyperplasia of myelopoiesis sprouts.

There are other criteria by which the doctor can draw a conclusion about the ongoing changes characteristic of polycythemia vera:

  1. Hepatosplenomegaly.
  2. Tendency to thrombosis.
  3. Increased sweating combined with weight loss, weakness.
  4. The presence of gene abnormalities, if genetic testing has been performed, when it comes to primary erythremia.
  5. Increase in the average amount of circulating blood.

All the criteria described above, except for the three main ones, which are considered large, are small. As for the major diagnostic criteria, this is an increase in the mass of circulating red blood cells, splenomegaly, supersaturation of arterial blood with oxygen. To establish a diagnosis, it is usually sufficient to have three of these major criteria, which are combined with two or three minor ones. Differential diagnosis is carried out by a hematologist between conditions that are accompanied by erythrocytosis - heart defects, tuberculosis, tumors, etc.

Treatment methods

The earlier a person seeks help, the more effective the therapy can be. At the third stage, or when layering another tumor process on erythremia, symptomatic therapy is performed in combination with chemotherapy treatment. Chemotherapeutic treatment may be recommended at other stages of the disease, but the body does not always give an adequate response to it. Of the symptomatic means that improve the quality of life, the following are used:

  1. Drugs against high blood pressure, mainly from the group of ACE inhibitors.
  2. Antihistamines for itching, skin irritation, and other allergic reactions.
  3. Antiplatelet agents and anticoagulants for blood thinning with a tendency to thrombosis.
  4. Local and systemic hemostatic agents for bleeding tissues.
  5. Medications to lower uric acid.

Treatment options for polycythemia vera may include:

  1. Bloodletting, or removing a small amount of blood from the bloodstream (phlebotomy). As a rule, they are done in volume (according to indications) and a break of 3-4 days in a course of several sessions. The blood after such manipulations becomes more liquid, but they cannot be done if there is a recent history of blood clots. Before treatment with bloodletting, the patient is given a solution of Reopoliglyukin, as well as Heparin.
  2. Erythrocytapheresis. It is used to clean the blood from excess red blood cells, as well as from platelets. Such sessions are done once a week.
  3. Chemotherapy. It is used, as a rule, when the disease reaches the tumor stage - the second B. Other indications for chemotherapy are the presence of complications from the peritoneal organs, the general plight of a person, and an increase in the number of all blood elements. For chemotherapy, or cytoreductive therapy, cytostatics, antimetabolites, alkylating drugs, biological drugs are used. The most commonly prescribed drugs are Leukeran, Hydroxyurea, Mielosan, recombinant interferon.
  4. Treatment of iron deficiency with androgens, erythropoietin, which are most often used in combination with glucocorticosteroids.
  5. Radiation therapy. It is used to irradiate the spleen area and stop the cancer process in it, it is used with a strong increase in the size of the organ.
  6. Transfusion of erythrocyte mass from purified erythrocytes. Used for severe anemia up to coma. If thrombocytopenia increases in the final stages of polycythemia vera, a platelet mass transfusion from a donor may be necessary.

Bone marrow transplantation for a disease such as erythremia often leads to adverse results, so it is rarely used. In some cases, splenectomy is indicated, but with the development of acute leukemia, such an operation is not performed even with severe splenomegaly.

Features of treatment in pregnant women

During pregnancy, this pathology occurs rarely. However, if there is a predisposition (hereditary or from secondary factors), pregnancy, childbirth and abortion can become a trigger for the development of polycythemia vera. Pregnancy always worsens the course of this disease, and its outcome may be more serious than outside of gestation. However, in 50% of cases, pregnancy ends in a successful birth. The remaining half is accounted for by miscarriages, developmental delay, anomalies in the structure of the fetal body.

Treatment of the disease in pregnant women is not easy. Most drugs are strictly contraindicated, as they have a pronounced teratogenic property. Therefore, during pregnancy, bloodletting therapy and, if necessary, glucocorticosteroids are mainly performed. To prevent complications and early detection of the disease in pregnant women, blood tests should be carried out regularly according to the schedule indicated by the observing obstetrician-gynecologist.

What Not to Do

It is strictly forbidden to use diuretics, which additionally thicken the blood. Also, in our time, the use of radioactive phosphorus preparations, which seriously inhibit myelopoiesis and often lead to the development of leukemia, is limited. Also, you can not save the old nutrition system: the diet must change. All foods that enhance blood formation, such as the liver, are banned. It is better to form a diet as a dairy-vegetable one, and refuse excess meat.

The patient should not overload the body, engage in heavy sports, ignore regular rest. Treatment with folk remedies can be used, but only after a thorough study of all the remedies by the doctor in terms of composition, in order to prevent an increase in the production of red blood cells. Most often, symptomatic therapy is used to remove uric acid, reduce pain and itching of the skin, etc.

Prevention and prognosis

Methods of prevention have not yet been developed. The prognosis for life varies depending on the severity of the course of the disease. Without treatment, up to a third of patients die within the first 5 years of diagnosis. If you carry out a full-fledged therapy, then you can extend the life of a person for a flight or more. The most common cause of death is thrombosis, and only occasionally do people die from blood cancer (leukemia) or heavy bleeding.

“Hemoglobin must be out of order” - we put ourselves a “diagnosis” and decide that we need to lean on products “with iron”

Hemoglobin- this is a protein with the help of which oxygen is transferred from the lungs to all tissues of the body, - explains Marina Georgievna. - Hemoglobin is found in erythrocytes - "red blood cells". It consists of two parts - a protein (globin) and an iron compound (heme). A general blood test allows, among other parameters, to evaluate the content of red blood cells, hemoglobin in the body. Its norm for men is g / l, for women g / l. Deviation of these parameters both downwards, when the level of hemoglobin in the blood falls below g / l, and upwards (hemoglobin content - more than 160 g / l) is a pathology and carries a certain danger to the body.

Moms, watch your hemoglobin!

Particularly unfavorable anemia affects the health of expectant mothers: in this case, both the woman and the fetus suffer. Anemia complicates the course of pregnancy, childbirth and the postpartum period, affects the development of the child. Against the background of a decrease in the level of hemoglobin in the blood of a woman, toxicosis of the second half of the period of bearing a baby may worsen, edema appears. It also increases the risk of having a baby prematurely. Anemia of pregnant women is also dangerous because during the birth of a peanut, weakness of labor activity may occur, and the amount of blood loss may increase. And in the postpartum period, the production of breast milk decreases.

The danger of erythrocytosis- in the thickening of the blood increased risk of constipation of blood vessels. The level of red blood cell hemoglobin above the norm can provoke myocardial infarction, stroke, thrombosis of the vessels of the extremities and other ailments.

What can be hidden behind the diagnosis of erythrocytosis?

Erythrocytosis is a pathological condition in which the number of red blood cells and hemoglobin in the blood increases. At the same time, the blood becomes viscous, which makes it difficult to move through the vessels and disrupts oxygen metabolism. If left untreated, irreversible changes may occur in the body.

Such a syndrome is not a separate disease, and occurs as a result of the body's adaptation to various abnormal processes. In some cases, erythrocytosis indicates a disease that provoked excessive production of red blood cells by hematopoietic organs. Therefore, before treating the symptom itself, you should find out the causes of its occurrence.

Erythrocytosis - what is it, types and types of syndrome

Human blood is 50-60% plasma, the remaining 50-40% are formed elements - cells that are in suspension and perform certain functions. The most numerous blood cells are erythrocytes, which are responsible for transporting oxygen to the tissues and removing carbon dioxide from them. The hemoglobin protein found in red blood cells gives blood its red color.

Normally, plasma contains a certain amount of red blood cells, which can fluctuate within the established limits depending on the age and physiological state of the person. If the indicators deviate upward, erythrocytosis (polycytopenia) is diagnosed. Such a condition can be physiological or pathological - resulting from somatic diseases or hereditary factors.

A reduced number of erythrocytes in the peripheral blood indicates erythropenia, which can occur with a decrease in the production of erythrocytes, as well as for physiological reasons - with an increase in the volume of the liquid fraction of the blood, while the number of formed cells remains the same.

The rate of erythrocytes at the rate of a million cells per liter of blood, i.e. 1x10 12 / l - table

Classification of erythrocytosis

Since erythrocytosis is not a separate disease, but only a sign of a pathological condition, this syndrome is usually classified depending on developmental factors. There are three types of erythrocytosis:

  1. Physiological - characterized by increased synthesis of red blood cells during adaptation of the body to a lack of oxygen. It develops in residents of highlands with rarefied air. This physiological state is also noted in newborns in the first few days after birth (neonatal syndrome).
  2. Relative (false) - an increase in erythrocytes per unit of blood is due to a decrease in plasma volume with a significant loss of fluid in the body. At the same time, the synthesis of blood cells in the bone marrow remains within the normal range.
  3. Absolute (true) - occurs in pathological conditions that stimulate increased production of red blood cells and their release into the bloodstream.

In turn, true erythrocytosis by origin is divided into primary (hereditary) and secondary - acquired. Let us consider in more detail all types of absolute erythrocytosis:

  1. Absolute Primary:
    • family - of unknown origin;
    • hereditary - transmitted in an autosomal recessive manner.
  2. Absolute secondary:
    • toxic;
    • stressful;
    • myogenic;
    • essential;
    • hypoxic;
    • paraneoplastic (tumor).

Causes, mechanisms and factors of development

Relative erythrocytosis, caused by a strong loss of fluid or the release of plasma from the vascular bed, is also called hemoconcentration. The main development factors are:

  • severe vomiting and diarrhea in infectious diseases and poisonings;
  • increased sweating;
  • burns of a large area of ​​the skin;
  • diabetic kathiocidosis;
  • Gaisbeck's syndrome (stress erythrocytosis, pseudopolycythemia) - observed in stage III-IV obesity;
  • necrotic skin lesions.

Among the primary absolute erythrocytosis, there are family pathologies of unknown origin, observed in residents of Chuvashia, Alaska and Yakutia, and hereditary methemoglobinopathies. Congenital pathologies are characterized by a gene mutation transmitted from one of the parents in an autosomal recessive manner. In children with such a genetic pathology, hemoglobin has an increased affinity for oxygen, as a result of which its deposition in tissue cells is difficult and hypoxia occurs. The body, trying to compensate for the lack of oxygen, begins to synthesize more red blood cells.

According to the mechanism of development, secondary absolute erythrocytosis can be characterized as expedient - caused by insufficient oxygen supply to tissues in various diseases (hypoxia), and inappropriate - associated with increased production of erythropoietin (the hormone responsible for the synthesis of red blood cells).

Most often, the following groups of diseases are the cause of the development of appropriate erythrocytosis:

  1. Endocrine. For diseases accompanied by an increased hormonal background, an accelerated course of metabolic reactions is characteristic, which leads to increased oxygen consumption. To compensate for the lack of oxygen, the body begins to rapidly produce red blood cells. Such diseases include:
    • diffuse toxic goiter;
    • adenoma;
    • teriotoxicosis.
  2. Hormonally active tumors. Neoplasms in the organs of the kidneys, spleen and pituitary gland stimulate the production of hormones that affect both the growth of the bone marrow and the output of red blood cells deposited in the spleen. Such tumors are formed in the following diseases:
    • Conn's syndrome;
    • Itsenko-Cushing's syndrome;
    • androsteroma;
    • corticodrosteroma;
    • pheochromocytoma and pheochromoblastoma;
    • ovarian tumors in women;
    • paraganglioma.
  3. Pulmonary. Pathological conditions in which the process of blood oxygen saturation in the lungs is disturbed cause hypoxia. The body's response to a lack of oxygen is an accelerated synthesis of red blood cells. Pulmonary erythrocytosis occurs with such diseases:
    • obstructive bronchitis;
    • asthma;
    • exudative pleurisy;
    • pulmonary tuberculosis;
    • pneumosclerosis;
    • emphysema;
    • Ayers-Arrilaga syndrome;
    • diffuse fibrosis.
  4. Cardiovascular. With pathologies of the heart, there is a violation of blood circulation, as a result of which the tissues are supplied with blood in an insufficient volume. The resulting circulatory hypoxia provokes an increased synthesis of red blood cells. These pathologies include:
    • congenital heart defects;
    • heart attack;
    • Tossig-Bing syndromes;
    • Guysbeck's syndrome;
    • stenosis of the vessels.
  5. Diseases of the gastrointestinal tract. As a result of increased absorption of vitamin B 12, which is actively involved in hematopoiesis, in such diseases:
    • pancreatic tumors;
    • stomach or duodenal ulcer;
    • hepatocarcinoma.

Factors that can provoke erythrocytosis include:

  • smoking;
  • carbon monoxide poisoning;
  • stress;
  • increased physical activity (myogenic erythrocytosis);
  • an overdose of vitamin B 12;
  • an overdose of drugs with glucocorticoids, as well as those containing nickel, magnesium, cobalt.

Symptoms

With secondary erythrocytosis, the syndrome develops gradually and in the first stages may not manifest itself outwardly, and also not be reflected in the person's well-being. Pathology can be suspected only when taking a blood test - it will be viscous, thick, and have a rich dark red color.

At the second erythremal stage, the following symptoms are noted:

  1. Reddening of the skin color, especially pronounced on the face and hands (does not apply to relative species).
  2. Cyanosis (bluish hue) is especially noticeable on the phalanges of the fingers, under the nail plate, in the nasolabial triangle, at the tip of the nose. It manifests itself in tissue hypoxia due to cardiovascular or pulmonary diseases. This symptom is also present in children with hereditary types of erythrocytosis.
  3. Thrombosis occurs in all types and forms of pathology, since it is caused by increased blood viscosity and a tendency to form blood clots.
  4. Enlargement of the spleen and liver (hepatosplenomegaly).
  5. Pain in the joints caused by the accumulation of purines in them.
  6. Angina pectoris and pain in the heart are caused by an increased load on the organ when pumping viscous blood.
  7. Increase in blood pressure.
  8. Skin itching caused by damage to microvessels.
  9. Headaches and mood changes are common in diseases of the endocrine system or pituitary tumors.

With prolonged absence of treatment, the third stage develops - the anemic stage, which is characterized by the growth of connective tissue in the bone marrow, which contributes to a decrease in hematopoietic cells and causes a decrease in the number of all formed elements. At this stage, which is also called terminal, the following symptoms are noted:

  • hemorrhagic hemorrhages provoked by a decrease in platelet synthesis;
  • bleeding gums;
  • pallor of the skin caused by a decrease in hemoglobin and red blood cells;
  • general weakness, increased fatigue;
  • fainting;
  • weight loss.

Diagnostics

To confirm the diagnosis, based on the clinical manifestations of erythrocytosis, laboratory tests and instrumental studies of internal organs are carried out.

A general blood test shows the number of formed elements, as well as the erythrocyte sedimentation rate. The results of such a study can confirm the suspicion of the syndrome and determine the stage of its development.

Erythrocytosis: treatments and diet

Erythrocytosis is a blood disease in which the number of red blood cells increases dramatically. This imbalance causes a decrease in blood viscosity and an increase in the load on the entire circulatory system. Most often, blood viscosity is increased in residents of high mountains, where the oxygen content in the air is reduced, respectively, more red blood cells are produced, and pathology develops.

Decreased blood viscosity in erythrocytosis

The danger of erythrocytosis is that the blood becomes much more viscous. Just imagine how quickly the pump fails, forced to pump syrup instead of water. But with increased blood viscosity, the load on the heart, the pump of our circulatory system, also sharply increases. Therefore, doctors prescribe in such cases antiplatelet agents - drugs that reduce blood viscosity (for example, aspirin). In addition, alternative medicine comes to the rescue.

In the treatment of erythrocytosis, along with medication, alternative methods such as hirudotherapy and apitherapy are used.

Hirudotherapy is a treatment method that uses medicinal leeches. A sucked leech injects a special substance into the human blood - hirudin, which acts instantly, and its effect lasts up to several days. For the treatment of blood viscosity, it is necessary to conduct hirudotherapy courses 1-2 times a year.

Apitherapy - methods of treating various diseases using live bees, as well as honey and other bee products. With erythrocytosis, a tincture of dead bees (that is, from bees that died a natural death) helps well. The chitinous cover of bees contains heparin, a special substance that regulates blood clotting, which in the human body is produced by liver cells.

In the treatment of increased blood viscosity, heparin and hirudin have a similar effect, contributing to rapid thinning. Well, since both of these substances are of natural origin, they are absolutely harmless and rarely cause allergies.

Nutrition for erythrocytosis: foods that reduce blood viscosity

Compliance with a diet for erythrocytosis is one of the prerequisites for a speedy recovery.

  • Try to limit your intake of foods rich in vitamin K, as they help blood clot and make it even thicker. Aronia, spinach, lettuce and other leafy vegetables are especially rich in this vitamin.
  • Make sure that your body regularly receives iodine: it reduces blood viscosity and increases vascular tone. Grind dry sea kale in a coffee grinder and season dishes with it instead of salt (at the rate of 1 teaspoon per day).
  • Eat foods that reduce blood viscosity and contain taurine, which normalizes blood pressure: poultry, white fish, shellfish, walnuts and almonds (preferably eat 1 tablespoon of nuts daily).
  • Eat fish at least 3-4 times a week, giving preference to fatty marine and ocean varieties: fish oil contains substances that contribute not only to thinning the blood, but also to the resorption of existing blood clots and sclerotic plaques.
  • Remember that blood viscosity is reduced by garlic, tomatoes, sweet bell peppers (especially red ones), cherries, cherries, melons, grapefruits, sweet clover grass, gingko biloba.
  • Include chili peppers in your diet: they contain vitamins A and E, as well as potassium and folic acid, which are great for thinning the blood and preventing cardiovascular disease.
  • Drink 120 ml of freshly squeezed orange juice daily. Among other things, it is the richest source of vitamin C. (However, it should be remembered that orange juice is contraindicated in stomach ulcers and gastritis with high acidity.)
  • An excellent blood thinner is 1 glass of dry red wine with lunch or dinner.

Physical education with erythrocytosis and treatment with folk remedies

With erythrocytosis, not only the diet should be changed, but also the motor regimen.

Physical education and sports have a beneficial effect on the body: the level of harmful lipids that increase blood viscosity (including cholesterol) decreases, and overall metabolism improves. In the course of clinical studies, it was found that during physical inactivity, the level of cholesterol and lipids exceeded the norm in almost half of the subjects. However, after 12 months of active sports, it remained only in 20% of the participants in the experiment, and a year later it reached a normal value in almost all.

It is possible to treat erythrocytosis and folk remedies using infusions and decoctions. The following are recipes for blood thinning.

Take 250 g of peeled garlic, add 300 g of honey. Mix thoroughly and infuse for 3 weeks. Take 1 tbsp. spoon 3 times a day 40 minutes before meals.

Pour 100 g of ground nutmeg into 0.5 liters of vodka. Infuse for 3 weeks, shaking daily, then strain. Take 3 times a day 30 minutes before meals, adding drops of tincture to 1/4 cup of hot water. When the tincture is over, take a break for 10 days. In total, it is desirable to conduct 5 courses of treatment.

Grind 1/2 teaspoon of dry flowers of bitter wormwood in a coffee grinder, add them to a glass of kefir and mix. Drink daily before bed. After 1 week, take a break for 7-10 days, after which the course of treatment is repeated. This wonderful remedy not only thins the blood, but also cleanses the liver and restores immunity.

Rinse thoroughly and grind 200 g of fresh mulberry roots (mulberry). Put in an enamel pan, pour 3 liters of cold water and leave for 1 hour. Then put on a slow fire and hold for 15 minutes after boiling, then remove from the stove, cool and strain. Within 5 days, take 200 ml 3 times a day before meals, then take a break for 2-3 days. To achieve the result, it is necessary to carry out 2-3 courses of treatment. Decoction must be stored in the refrigerator.

Grate fresh ginger root (about 4 cm in size), add 1 pinch of cinnamon and 1 teaspoon of green tea. Mix, pour 0.5 liters of boiling water. Infuse for 45 minutes, then strain, add % lemon juice and honey to taste. Drink during the day.

Diet for anemia and erythrocytosis

The skin is pale, dizzy, headaches, tinnitus, shortness of breath, palpitations when walking, and then weakness - you feel like a squeezed lemon, you get tired quickly .. "Hemoglobin must be out of order" - we put ourselves “diagnose” ourselves and decide that we need to lean on products “with iron”

And at best, we eat more apples a day. Whereas iron deficiency anemia should be taken seriously!

Deviation is serious

Hemoglobin is a protein with the help of which oxygen is transferred from the lungs to all tissues of the body, - Marina Georgievna explains. - Hemoglobin is found in erythrocytes - "red blood cells". It consists of two parts - a protein (globin) and an iron compound (heme). A general blood test allows, among other parameters, to evaluate the content of red blood cells, hemoglobin in the body. Its norm for men is g / l, for women g / l. Deviation of these parameters both downwards, when the level of hemoglobin in the blood falls below g / l, and upwards (hemoglobin content - more than 160 g / l) is a pathology and carries a certain danger to the body.

It is not worth ignoring the above symptoms also because any anemia exacerbates existing diseases. If something is bothering you, consult a doctor: he will give a referral for a blood test, and, if necessary, prescribe treatment.

Mommy, watch your hemoglobin!

Anemia has an especially unfavorable effect on the health of expectant mothers: in this case, both the woman and the fetus suffer. Anemia complicates the course of pregnancy, childbirth and the postpartum period, affects the development of the child. Against the background of a decrease in the level of hemoglobin in the blood of a woman, toxicosis of the second half of the period of bearing a baby may worsen, edema appears. It also increases the risk of having a baby prematurely. Anemia of pregnant women is also dangerous because during the birth of a peanut, weakness of labor activity may occur, and the amount of blood loss may increase. And in the postpartum period, the production of breast milk decreases.

However, elevated levels of hemoglobin and red blood cells - erythrocytosis - are just as serious as anemia.

The danger of erythrocytosis is in the thickening of the blood, the increased risk of constipation of blood vessels. The level of red blood cell hemoglobin above the norm can provoke myocardial infarction, stroke, thrombosis of the vessels of the extremities and other ailments.

Polycythemia (erythremia, Wakez's disease): causes, signs, course, therapy, prognosis

Polycythemia is a disease that can be assumed just by looking at the patient's face. And if you still conduct the necessary blood test, then there will be no doubt at all. In reference books, it can also be found under other names: erythremia and Wakez's disease.

Redness of the face is quite common and there is always an explanation for this. In addition, it is short-term and does not linger for a long time. Various reasons can cause sudden reddening of the face: fever, high blood pressure, hot flashes during menopause, a recent tan, an awkward situation, and emotionally labile people tend to blush often, even if others do not see any prerequisites for this.

Polycythemia is different. Here, the redness is persistent, not transient, evenly distributed over the entire face. The color of an overly “healthy” plethora is saturated, bright cherry.

What kind of disease is polycythemia?

True polycythemia (erythremia, Wakez's disease) belongs to the group of hemoblastoses (erythrocytosis) or chronic leukemia with a benign course. The disease is characterized by the growth of all three hematopoiesis with a significant advantage of erythrocyte and megakaryocytic, due to which there is an increase not only in the number of red blood cells - erythrocytes, but also other blood cells that originate from these germs, where the source of the tumor process is the affected precursor cells of myelopoiesis. It is they who begin uncontrolled proliferation and differentiation into mature forms of erythrocytes.

Most affected under such conditions are immature red blood cells, which are hypersensitive to erythropoietin even at low doses. With polycythemia, the growth of leukocytes of the granulocytic series (primarily stab and neutrophils) and platelets. The cells of the lymphoid series, which include lymphocytes, are not affected by the pathological process, since they come from a different germ and have a different way of reproduction and maturation.

Cancer or not cancer?

Erythremia - not to say that it occurs all the time, however, there are a couple of people in a town of 25 thousand people, while for some reason men of 60 or so “love” this disease more, although any person can meet with such a pathology age. True, for newborns and young children, true polycythemia is absolutely not typical, so if erythremia is found in a child, then most likely she will wear secondary character and be a symptom and consequence of another disease (toxic dyspepsia, stress erythrocytosis).

For many people, a disease classified as leukemia (whether acute or chronic) is primarily associated with blood cancer. Here it is interesting to understand: is it cancer or not? In this case, it would be more expedient, clearer and more correct to talk about the malignancy or benignity of true polycythemia in order to determine the boundary between "good" and "evil". But, since the word "cancer" refers to tumors from epithelial tissues, then in this case this term is inappropriate, because this tumor comes from hematopoietic tissue.

Wakez disease refers to malignant tumors, but it is characterized by high differentiation of cells. The course of the disease is long and chronic, for the time being qualified as benign. However, such a course can only last up to a certain point, and then with proper and timely treatment, but after a certain period of time, when significant changes in erythropoiesis occur, the disease becomes acute and acquires more “evil” features and manifestations. Here it is - true polycythemia, the prognosis of which will completely depend on how quickly its progression occurs.

Why do sprouts grow incorrectly?

Any patient suffering from erythremia, sooner or later asks the question: "Why did this" illness happen "to me?". The search for the cause of many pathological conditions is usually useful and gives certain results, increases the effectiveness of treatment and promotes recovery. But not in the case of polycythemia.

The causes of the disease can only be assumed, but not definitely stated. Only one clue can be for the doctor when determining the origin of the disease - genetic abnormalities. However, the pathological gene has not yet been found, so the exact localization of the defect has not yet been determined. There are, however, suggestions that Wakez's disease may be associated with trisomy 8 and 9 pairs (47 chromosomes) or other violation of the chromosomal apparatus, for example, the loss of a segment (deletion) of the long arm C5, C20, but this is still guesswork, although built on conclusions of scientific research.

Complaints and clinical picture

If there is nothing to say about the causes of polycythemia, then we can talk about clinical manifestations for a long time and a lot. They are bright and varied, since already from the 2nd degree of development of the disease, literally all organs are drawn into the process. Subjective sensations of the patient are of a general nature:

  • Weakness and constant feeling of fatigue;
  • Significant decrease in performance;
  • increased sweating;
  • Headaches and dizziness;
  • Marked memory loss;
  • Visual and auditory disorders (decrease).

Complaints characteristic of this disease and characterized by it:

  • Acute burning pain in the fingers and toes (the vessels are clogged with platelets and red blood cells, which form small aggregates there);
  • Soreness, though not so burning, in the upper and lower limbs;
  • Itching of the body (a consequence of thrombosis), the intensity of which increases markedly after a shower and a hot bath;
  • Periodic appearance of a rash such as urticaria.

It's obvious that cause all these complaints microcirculation disorder.

reddening of the skin with polycythemia

As the disease progresses, more and more new symptoms are formed:

  1. Hyperemia of the skin and mucous membranes due to the expansion of capillaries;
  2. Pain in the region of the heart, resembling angina pectoris;
  3. Pain in the left hypochondrium caused by overload and enlargement of the spleen due to the accumulation and destruction of platelets and red blood cells (it is a kind of depot for these cells);
  4. Enlargement of the liver and spleen;
  5. Peptic ulcer of the stomach and 12 duodenal ulcer;
  6. Dysuria (difficulty urinating) and pain in the lumbar region due to the development of uric acid diathesis, which was caused by a shift in blood buffer systems;
  7. Pain in bones and joints as a result hyperplasia(overgrowth) bone marrow;
  8. Gout;
  9. Manifestations of hemorrhagic nature: bleeding (nasal, gingival, intestinal) and skin hemorrhages;
  10. Injections of the vessels of the conjunctiva, which is why the eyes of such patients are called "rabbit eyes";
  11. Telangiectasias;
  12. Tendency to thrombosis of veins and arteries;
  13. Varicose veins of the lower leg;
  14. Thrombophlebitis;
  15. Possible thrombosis of the coronary vessels with the development of myocardial infarction;
  16. Intermittent claudication, which may result in gangrene;
  17. Arterial hypertension (almost 50% of patients), giving rise to a tendency to strokes and heart attacks;
  18. Respiratory damage due to immune disorders, which cannot adequately respond to infectious agents that cause inflammation. In this case, red blood cells begin to behave like suppressors and suppress the immunological response to viruses and tumors. In addition, they are in the blood in an abnormally high amount, which further aggravates the state of the immune system;
  19. The kidneys and urinary tract suffer, so patients have a tendency to pyelonephritis, urolithiasis;
  20. The central nervous system does not remain aloof from the events taking place in the body, when it is involved in the pathological process, symptoms of cerebrovascular accident, ischemic stroke (with thrombosis), hemorrhage (less often), insomnia, memory impairment, mnestic disorders appear.

Asymptomatic to end stage

Due to the fact that polycythemia in the early stages is characterized by an asymptomatic course, the above manifestations do not occur in one day, but accumulate gradually and for a long time, it is customary to distinguish 3 stages in the development of the disease.

Initial stage. The patient's condition is satisfactory, the symptoms are moderately severe, the duration of the stage is about 5 years.

Stage of developed clinical manifestations. It takes place in two stages:

II A - proceeds without myeloid metaplasia of the spleen, subjective and objective symptoms of erythremia are present, the duration of the period is years;

II B - myeloid metaplasia of the spleen appears. This stage is characterized by a clear picture of the disease, the symptoms are pronounced, the liver and spleen are significantly enlarged.

The terminal stage, which has all the signs of a malignant process. Complaints of the patient are diverse, "everything hurts, everything is wrong." At this stage, cells lose their ability to differentiate, which creates a substrate for leukemia, which replaces chronic erythremia, or rather, it turns into acute leukemia.

The terminal stage is characterized by a particularly severe course (hemorrhagic syndrome, rupture of the spleen, infectious and inflammatory processes that cannot be treated due to deep immunodeficiency). It usually ends in death soon after.

Thus, the life expectancy for polycythemia is years, which may be not bad, especially considering that the disease can overtake after 60. And this means that there is a certain prospect of living up to 80 years. However, the prognosis of the disease still depends most of all on its outcome, that is, on what form of leukemia erythremia is transformed into at stage III (chronic myeloid leukemia, myelofibrosis, acute leukemia).

Diagnosis of Wakez disease

The diagnosis of polycythemia vera is mainly based on laboratory data with the determination of the following indicators:

  • Complete blood count, in which you can notice a significant increase in red blood cells (6.0-12.0 x / l), hemoglobin (g / l), hematocrit (ratio of plasma and red blood). The number of platelets can reach levels of 10 9 /l, while they can significantly increase in size, and leukocytes - up to 9.0-15.0 x 10 9 /l (due to rods and neutrophils). ESR with true polycythemia is always reduced and can reach zero.

Morphologically, erythrocytes do not always change and often remain normal, but in some cases, erythremia can be observed anisocytosis(erythrocytes of different sizes). Platelets indicate the severity and prognosis of the disease with polycythemia in the general blood test (the more there are, the more severe the course of the disease);

  • BAC (biochemical blood test) with the determination of the level of alkaline phosphatase and uric acid. For erythremia, the accumulation of the latter is very characteristic, which indicates the development of gout (a consequence of Wakez's disease);
  • Radiological examination using radioactive chromium helps to determine the increase in circulating red blood cells;
  • Sternal puncture (bone marrow sampling from the sternum) followed by cytological diagnosis. In the preparation hyperplasia of all three germs with a significant predominance of red and megakaryocytic;
  • Trepanobiopsy(histological examination of material taken from the ilium) is the most informative method that allows you to most reliably identify the main symptom of the disease - three-growth hyperplasia.

In addition to hematological parameters, to establish the diagnosis of polycythemia vera, the patient is sent for an ultrasound examination (ultrasound) of the abdominal organs (enlargement of the liver and spleen).

So, the diagnosis is established ... What's next?

And then the patient is waiting for treatment in the hematological department, where tactics are determined by clinical manifestations, hematological parameters and the stage of the disease. Therapeutic measures for erythremia usually include:

  1. Bloodletting, which allows to reduce the number of red blood cells to 4.5-5.0 x / l and Hb (hemoglobin) to 150 g / l. To do this, with an interval of 1-2 days, 500 ml of blood is taken until the number of erythrocytes and Hb drops. The bloodletting procedure is sometimes replaced by hematologists with erythrocytopheresis, when, after sampling by centrifugation or separation, red blood is separated, and the plasma is returned to the patient;
  2. Cytostatic therapy (myelosan, imifos, hydroxyurea, hydroxycarbamide);
  3. Antiplatelet agents (aspirin, dipyridamole), which, however, require caution in their use. So, acetylsalicylic acid can enhance the manifestation of hemorrhagic syndrome and cause internal bleeding if the patient has a stomach ulcer or duodenal ulcer;
  4. Interferon-α2b, which is successfully used with cytostatics and increases their effectiveness.

The treatment regimen for erythremia is signed by the doctor for each case individually, so our task is only to briefly acquaint the reader with the drugs used to treat Wakez's disease.

Nutrition, diet and folk remedies

A significant role in the treatment of polycythemia is assigned to the regime of work (reduction of physical activity), rest and nutrition. In the initial stage of the disease, when the symptoms are not yet expressed or are weakly manifested, the patient is assigned table number 15 (general), however, with some reservations. The patient is not recommended to consume foods that enhance hematopoiesis (liver, for example) and are offered to revise the diet, giving preference to dairy and vegetable products.

In the second stage of the disease, the patient is assigned table number 6, which corresponds to the gout diet and limits or completely excludes fish and meat dishes, legumes and sorrel. Having been discharged from the hospital, the patient must adhere to the recommendations given by the doctor during outpatient observation or treatment.

Question: "Is it possible to treat folk remedies?" sounds with the same frequency for all diseases. Erythremia is no exception. However, as already noted, the course of the disease and the life expectancy of the patient depend entirely on timely treatment, the purpose of which is to achieve a long and stable remission and delay the third stage for the longest possible time.

During the calm period of the pathological process, the patient must still remember that the disease can return at any time, therefore, he must discuss his life without exacerbation with the attending physician, in whom he is observed, periodically take tests and undergo an examination.

Treatment of blood diseases with folk remedies should not be generalized, and if there are many recipes to increase hemoglobin levels or to thin the blood, this does not mean at all that they are suitable for the treatment of polycythemia, for which, in general, medicinal herbs have not yet been found. Wakez's disease is a delicate matter, and in order to control the function of the bone marrow and thus affect the hematopoietic system, you need to have objective data that can be assessed by a person with certain knowledge, that is, the attending physician.

In conclusion, I would like to say a few words to readers about relative erythremia, which cannot be confused with true erythremia, since relative erythrocytosis can occur against the background of many somatic diseases and successfully end when the disease is cured. In addition, erythrocytosis as a symptom can accompany prolonged vomiting, diarrhea, burn disease and hyperhidrosis. In such cases, erythrocytosis is a temporary phenomenon and is associated primarily with dehydration of the body, when the amount of circulating plasma, which consists of 90% water, decreases.

Elevated red blood cells

Human blood is heterogeneous in its composition. Its liquid part (plasma) contains cells, the most numerous of which are erythrocytes. There are 4 to 5 million of them in every milliliter of blood.

Erythrocytes are blood components that play one of the most important functions in maintaining the vital activity of the whole organism. They are called red blood cells, these are cells in which there are no nuclei, but at the same time they contain proteins and fats, but most importantly hemoglobin. They make up about a quarter of all cells in the body. Our body creates about 2.3 million new red blood cells every second.

The main task of erythrocytes in our body is to deliver oxygen to the organs and tissues of the body, as well as to transport carbon dioxide from organs to the lungs. The erythrocyte contains a large amount of hemoglobin (up to 95%), while proteins and lipids occupy the remaining 5%. Outwardly, the erythrocyte resembles a disc, concave on both sides. Due to this shape, they have a slightly larger area, which improves the efficiency of gas exchange.

The size of erythrocytes is about 7-8 microns, which approximately coincides with the diameter of blood vessels. Blood cells enter the capillaries due to the fact that they can change their shape, this can explain the high speed of movement of red blood cells throughout the body.

Erythrocytes perform two main functions and a number of auxiliary ones. The main ones include:

  • oxygen transport,
  • transport of carbon dioxide.
  • Support for the ionic composition of the blood,
  • Participation in water-salt metabolism,
  • Adsorption of toxins, hormones and proteins,
  • Participation in thrombosis, as well as many others.

The main functions of erythrocytes are provided due to their shape, thereby increasing the area on which a sufficient amount of gas can be placed. Red blood cells can bind antigens and toxins on their surface, thereby reducing the effects of intoxication. Red blood cells live for about 120 days and complete their life cycle in the spleen.

The basis of the erythrocyte is hemoglobin, which, in turn, consists of the protein globin and the second component - the red pigment (heme), which contains iron. It is the gems that give the blood its well-known color. But the main thing is that thanks to the iron they contain, hemoglobin is able to capture oxygen, keep it in the blood and deliver it to the cells of the body for respiration.

The cellular structure of erythrocytes is such that they are not capable of independent reproduction, since they lack a nucleus with hereditary information encoded there. Their reproduction is carried out by the red bone marrow, which is located in the voids of the spongy bone tissue of the bones of the skeleton. The framework of the bone marrow is formed by reticular connective tissue. Attached to the fibers of this scaffold are stem cells specialized in the production of blood cells.

Preparations of future blood cells (staminal cells) are generated during the division of stem cells. What kind of blood cells the staminal cells will become determines the mechanism of quantitative regulation of hematopoiesis, the work of which depends on the needs of the body. Separated from the fibers of the connective tissue of the bone marrow, staminal cells immediately determine their purpose, having received an order from the body through special substances - hormones, and change in this direction.

Almost fully mature cells enter the bloodstream and soon become fully functional. The life span of red blood cells in blood vessels is 2 to 4 months. After that, the erythrocytes that have served their purpose will be sent to the spleen for disposal. Iron from them will be extracted and directed to the creation of new red blood cells. Every second, 2 million red blood cells are removed from the bloodstream, but the same number of new ones leave the bone marrow at the same time.

The norms of erythrocytes in human blood

Rates may vary based on age as well as gender differences.

A general blood test, as well as a quantitative indicator of erythrocytes, their shape and color helps to conduct a primary diagnosis. The number of red blood cells is a kind of health test. Their number can be either more or less, which is a kind of signal about violations in the body. The reasons for this can be both respiratory or circulatory disorders of the body, and all kinds of pathologies of the blood system, digestive system and other quite dangerous diseases. The size and shape of red blood cells may change:

  • Microcytosis volume reduction in hemolytic anemias or malignant neoplasms;
  • Macrocytosis increase in size with B12 or folic acid anemia, with pulmonary or hepatic pathology;
  • Megacytosis of huge (giant) red blood cells, which can indicate such dangerous diseases as acute leukemia or severe anemia.
  • Poikilocytosis of an irregularly shaped cell, which indicates violations of regeneration processes as a result of anemia.

What are the causes of erythrocytosis

The process of production of blood cells is very complex and is associated with the functioning of many body systems. We can talk about dysfunctions in the work of various organs, hormonal disorders, as well as disorders of the nervous system. The results of recent studies of the hematopoietic system show that the causes of polycythemia lie at the cellular and molecular level. The relationship between the onset of the disease and the mutation of one of the enzymes that make up stem cells has been traced.

  • Respiratory system disorders (bronchitis, pneumonia);
  • Anomalies in the development of the heart (heart defects);
  • vitamin deficiency;
  • The use of low-quality water;
  • Erythremia is a condition in which all indicators associated with red blood cells in the blood increase.
  • Infectious diseases;
  • Being in conditions of low oxygen content;
  • Oncological processes;
  • Pathological processes in the cells-sprouts of the bone marrow;
  • Complications of treatment of various diseases;
  • Insufficient amount of enzymes for the digestion of food, which disrupts the biochemical processes in the body;
  • Increase in blood viscosity.

If the level of erythrocytes rises to 8-12 * 1012 / l, then one can judge the occurrence of such dangerous conditions as erythremia or acute leukemia. Under conditions of low oxygen content, compensatory erythrocytosis develops. Erythrocytes can also be increased with pulmonary or heart failure, as well as with increased blood viscosity, for example, with dehydration, diarrhea.

If the analysis shows a normal color index (0.85-1.05) and an increase in the number of red blood cells, then iron deficiency anemia and malignant diseases should be excluded. Also, these diseases should be excluded in case of a decrease in the color index below 0.8.

Excess red blood cells

The quantitative composition of blood cells can be determined by separating them from plasma by rotating a container with blood taken for analysis in a centrifuge. A decrease in the number of red blood cells in the blood from the norm indicates anemia, and their excess is called erythrocytosis.

Erythrocytosis is dangerous because the blood becomes thick and viscous. At the same time, the risk of blood clots and, as a result, blockage of blood vessels increases many times over. It is very difficult for the heart to pump a viscous fluid and it wears out quickly. The speed of blood flow in blood vessels that have expanded under pressure slows down. Viscous blood hardly penetrates into the thinnest capillaries.

The blood supply of some organs becomes insufficient, while others, on the contrary, become overfilled with blood, which leads to dysfunctions in their work. Among the pathologies associated with erythrocytosis are duodenal ulcer and stomach ulcer, enlarged spleen and liver, thrombosis, internal bleeding, joint pain, heart failure, constant itching of the skin.

Erythrocytoses are of two types, depending on the nature of their occurrence:

  • Compensatory, when the hematopoietic system adapts to the body's needs for increased oxygen supply. This happens when a person enters high-mountainous regions for a long period, with constant significant physical exertion, with respiratory diseases, hypertension.
  • True polycythemia, when an increase in the number of red blood cells is caused by a malfunction of the body systems involved in hematopoiesis.

The consequences of erythrocytosis can be such conditions as:

  • Increase in the size of parenchymal organs;
  • Violation of the work of organs and tissues;
  • Thickening of the blood, which can lead to increased thrombosis.

Ways to reduce blood viscosity

The currently existing methods of treating the disease are mainly in attempts to reduce the negative consequences of the overproduction of red blood cells - increased blood viscosity, thrombosis, bleeding.

  1. The main method of treating erythrocytosis at present is the removal of part of the circulating blood from the body by bloodletting in combination with the introduction into the circulatory system of a solution that replaces the blood plasma and drugs that prevent its coagulation.
  2. A more advanced method of treating erythrocytosis, also associated with the selection of blood from the patient, is erythrocytapheresis. In this case, the blood is processed in the apparatus, where excess red blood cells are removed from it. After this treatment, the blood returns to the patient's circulatory system.
  3. Taking anticoagulants and antiplatelet agents - a group of drugs that suppress the ability of blood to clot and form blood clots and, ultimately, reduce blood viscosity.
  4. Treatment of erythrocytosis with leeches - hirudotherapy. A leech that has reached the human circulatory system reduces the ability of blood to clot by injecting into it the substance it produces - hirudin.
  5. Treatment of erythrocytosis with the help of bees and bee products - apitherapy. Well regulates blood clotting tincture on dead bees due to heparin, extracted from the chitinous cover of dead insects.

erythremia

General description of the disease

Erythremia (otherwise Wakez's disease or polycythemia) is a disease of the human hematopoietic system of a chronic nature, during which the amount of red blood cells is increased in the bone marrow.

Erythremia is considered a disease of adults (age category from 40 to 60 years), and predominantly men are ill. The disease is very rare in children.

The causes of this disease have not yet been identified. In order to diagnose erythremia, it is necessary to do a blood test, to obtain more detailed information on the number and content of leukocytes, a bone marrow biopsy is done. Also, there is an increase in hemoglobin levels and an increase in blood viscosity.

Polycythemia occurs in three stages.

At each stage of the disease, different symptoms appear.

  1. 1 Initial stage. Erythremia begins its manifestation with increased fatigue, dizziness, noise and a feeling of heaviness in the head, itching may disturb and there may be slight reddening of the skin. At the same time, there is a sleep disorder, mental abilities decrease, limbs constantly vegetate. There are no outward signs of Wakez disease at this stage.
  2. 2 Expanded. At this stage, the patient is tormented by severe headaches (often similar to migraine attacks), pain in the heart area and bones, pressure is almost always increased, there is a strong exhaustion of the body, which causes severe weight loss, deterioration in hearing and visual abilities, increases in the volume of the spleen. Distinctive features are the redness of the mucous membranes of the palate, tongue and conjunctiva, the skin acquires a red-bluish tint. Blood clots and ulcers occur, bruises appear with the least injuries, and severe bleeding is observed when teeth are removed.
  3. 3 Terminal. If you do not carry out therapeutic measures, then due to blockage of blood vessels, an ulcer of the duodenum, stomach, cirrhosis of the liver, acute leukemia and myeloid leukemia can form.

Useful products for erythremia

To combat polycythemia, the patient should adhere to a vegetable and sour-milk diet. Recommended for use:

  • raw, boiled, stewed vegetables (especially beans);
  • kefir, yogurt, cottage cheese, milk, yogurt, sourdough, fermented baked milk, sour cream (required without fillers, better home-made);
  • eggs;
  • greens (spinach, sorrel, dill, parsley);
  • dried apricots and grapes;
  • dishes made from whole grains (tofu, brown rice, whole grain bread);
  • nuts (almonds and Brazil nuts);
  • tea (especially green).

Traditional medicine for erythremia

For treatment, the use of leeches and bloodletting (phlebotomy) is indicated. These treatments help to lower the iron content in the body, which helps to normalize the number of red blood cells in the blood. The frequency and duration of such procedures depends on the stage of erythremia. These methods can only be used when prescribed by medical professionals and in their direct presence.

In order not to form blood clots, you need to move more and spend time in the fresh air. Also, juice made from chestnut (horse) flowers will help get rid of thrombosis.

To normalize blood pressure, sleep, migraine, you should drink an infusion of medicinal sweet clover. It should be noted that the course of treatment should not be more than a day.

To expand blood vessels, improve blood flow, increase the resistance of capillaries and blood vessels, you need to drink decoctions of periwinkle, nettle, hornbeam grass and burial ground.

Polycythemia is a condition of the body in which the production of red blood cells increases pathologically. Often this is associated with an increase in total blood volume and spleen, the number of leukocytes and platelets is also increased. There is a gender predisposition: men are less resistant to the disease, they get sick several times more often than the female half of the population. The most vulnerable period is the middle and older age, but polycythemia is increasingly being recorded in newborns.

Classification and symptoms

The classification of polycythemia is quite detailed. It is customary to single out not only the forms, but also the stages of the disease. This allows you to more accurately prescribe a course of therapy and rehabilitation. There are several forms of the disease:

  • polycythemia vera (primary, Wakez's disease). The erythroblastic germ in the bone marrow undergoes hyperplastic changes.
  • relative polycythemia (secondary, Gaisbeck's syndrome).

Additionally, polycythemia vera is divided into 3 stages:

  • primary. It develops at the onset of the disease, the duration of the course may exceed a five-year period. The manifestation of symptoms is mild or there is no symptomatic picture at all;
  • expanded. It may take up to 20 years. Occurs with and without damage to the spleen.
  • heavy. It is characterized by the formation of oncological tumors of any localization, but more often not only the spleen, blood, but also the liver suffers.

Secondary polycythemia (Gaisbeck's syndrome) can develop as a reaction to prolonged stress, poor working conditions and lack of proper rest. Such a course can pass with a minimum therapeutic course, but subject to the eradication of the root cause of the disease. True polycythemia may be false. It does not lead to a shift in the erythrocyte, leukocyte, platelet balance.

The reasons

The pathogenesis of the disease largely depends on the form of the disease. It has been noted that polycythemia vera develops due to gene mutations, hereditary predisposition to distortion of hematopoietic processes. Pathology occurs with prolonged and significant hypoxia. All oncological processes that have formed in the bone marrow can have a detrimental effect on the natural and well-formed mechanism for the production of red blood cells. Secondary polycythemia can develop as a result of:

  • previous obstructive pulmonary disease;
  • pulmonary hypertension;
  • long-term failure of the heart;
  • oxygen starvation of the kidneys, as the main organ of the excretory system;
  • unfavorable climate, especially in high mountains;
  • oncological process of any localization;
  • exposure to infectious agents;
  • some features of labor (miners, high-altitude workers, etc.);
  • unhealthy environmental conditions (living in megacities, near hazardous industries, near major highways);
  • malicious smoking;
  • belonging to a European nation. Blame the prevailing genotype.

Many of the causes of the symptoms of the disease have not been studied. In particular, neonatal polycythemia occurs as a result of a violation of placental activity. The baby takes over the disease from the mother, while still in her womb. The transmission mechanism of the disease is poorly understood by doctors. Today, measures are being worked out to prevent this method of transmission of the disease.

Symptomatic picture

The first signs (syndrome) of the disease usually do not cause suspicion. In the early stages, a paroxysmal headache, visual impairment, a person loses sleep, complains of dizziness, nausea. The fingertips, both on the upper and lower limbs, are cold. Their temperature is practically independent of the ambient temperature.

In the expanded stage, muscle pain appears. The joints "ache" almost around the clock. The general condition worsens, working capacity, stress resistance, the ability to memorize large amounts of new information fall. The skin becomes bruised, the gums begin to bleed, nosebleeds can occur, provoked by minimal trauma to the mucosa. The spleen and liver are often enlarged. These are non-specific symptoms, but there are also specific signs. These include:

  • severe itching. Moreover, itching increases after any skin contact with water. Sometimes, it becomes unbearable, which leads to neurotic states;
  • in the pads on the fingers there is a burning sensation of varying intensity;
  • the skin of the face becomes red with a bluish tint;
  • saphenous veins are visualized;
  • persistent essential hypertension often occurs;
  • lips, tongue, whites of the eyes are filled with blood;
  • general weakness continues to progress, it is characteristic of the disease;
  • the risk of thrombosis, heart attacks, strokes increases sharply;
  • liver tissue changes according to the type of cirrhosis, the organ increases in size.

In the last stage of the disease, when uncontrolled growth of tumors leads to a critical drop in body weight and general intoxication, the entire symptomatic picture is pronounced. Anemia increases, the risk of gastric, uterine, intestinal bleeding increases. In the absence of competent treatment, or in case of detection of the disease in a late stage, a fatal outcome occurs.

Polycythemia in newborns

Neonatal polycythemia manifests itself 6-7 days after birth. More often, symptoms appear in full-term and large twins. The skin is painful, even a slight touch causes the child to cry. A reddish color of the skin appears, itching, body weight drops. Primary symptoms (manifestations) are confirmed by laboratory tests: erythrocytes, leukocytes and platelets significantly exceed the age norm. The liver and spleen are enlarged.

Diagnostic measures and prognosis

As soon as the primary signs of the disease are suspected, a qualitative diagnosis is necessary. It begins with a detailed history taking. Find out a possible hereditary origin, which requires a "family" history. Further, laboratory tests are prescribed: a general analysis of blood and urine. If deviations from the norm are detected, then the blood is examined for biochemistry and the leukocyte formula is determined.

In order to confirm or refute the diagnosis, it is necessary to conduct a histology and morphology of bone marrow punctate. A cytological examination will also be required. The causes and degree of damage to the body during the disease will help to identify ultrasound of the internal organs and blood vessels, FGDS, echocardiography, etc. You may need to consult related specialists: urologist, pulmonologist, gynecologist. The hematologist resolves the issue of dispensary registration.

Forecast

The prognosis of the disease depends on many factors: the severity and malignancy of the pathology, the correct treatment, the age of the patient, the individual capabilities of the body, etc. Only 1% of polycythemia transforms into leukemia, and up to 15% of patients need cytostatics. But the disease is not prone to self-healing or long-term "spontaneous" remissions. Lifelong follow-up with a hematologist is required. In severe cases, disability is assigned, it is typical for many patients with a similar diagnosis. In order for the prognosis to become more favorable, it is necessary to identify the symptoms in time and take full courses of treatment, carefully following medical prescriptions.

Treatment of polycythemia

To prescribe the right method of therapy, it is necessary to diagnose the exact cause of the disease. With true polycythemia, it is necessary to influence neoplasms in the red bone marrow, with secondary (Gaisbeck's syndrome), the cause that caused the disease is eliminated. Therapy for polycythemia vera is a rather lengthy and complex process. Therefore, there must be adequate treatment that affects the tumor and prevents its metastasis. When choosing medicines, the age of the patient must be taken into account, not all drugs are equally good for young and elderly patients.

Medical treatment

Therapy is aimed at eliminating the symptoms caused by the disease. With high blood pressure, antihypertensive drugs (enalapril, captopril, coverex) are recommended. Antihistamines are used to relieve itching (Fenkarol, Suprastinex) and other body responses. Antiplatelet agents (Vixipin, aspirin-based drugs) are used to reduce blood clotting.

Anticoagulants are prescribed to reduce the risk of blood clots. This pharmacological group includes Heparin, Worfarin, Dalteparin and antiplatelet drugs. When bleeding, it is appropriate to prescribe hemostatic agents. These include Vikasol, Epsilon-aminocaproic acid, Hemofobrin. Taking diuretics is strictly prohibited, they thicken the blood even more and cause various complications.

The use of interferons and tranquilizers

Interferons are specific proteins produced by the immune system. They are aimed at combating pathogenic flora, destroying the DNA and RNA of pathogens. Modern pharmacologists have created artificial interferons with a similar property, because drugs in this group improve the prognosis and reduce the signs of blood disease. The most commonly shown application is:

  • Interferon;
  • Interlock;
  • Diaferon;
  • Inferon;
  • Leukinferon;
  • Nazoferon;
  • Roferon;
  • Lfarekin;
  • Wellverona;
  • Introna A;
  • Sveferon.

Separately, I would like to say about tranquilizers in the treatment of Wakez's disease. Tranquilizers are drugs that have a calming effect. They are indicated if complex diagnostics, bloodletting is needed. Or there is itching, there is a fear of chemotherapy. In such cases, the use of Lorazepam, Ozepam, Medazepam (rudotel), Alprazolam (Xanax), Chlordiazepoxide (Elenium), Gidazepam, Diazepam (Valium), Seduxen, Relanium, Sibazon is indicated.

Chemotherapy

In cases where the disease progresses, a serious condition of the patient is observed, complications from the internal organs and blood vessels join, then chemotherapy is recommended. The drugs used in this case have a detrimental effect on tumor cells. Apply Hydroxyurea, Anagrelide and Bisulfan. The treatment process should be complex, therefore, drug therapy and prevention are used.

Non-drug therapy: briefly

This type of treatment is used in combination with medications. Only an integrated approach will provide a positive prognosis for recovery. You can not neglect any opportunity to stop the progression of the disease, otherwise it will be very difficult to achieve remission. Here are the most useful methods.

diet therapy

Meat and fish are left in the diet at the very least. But their fatty varieties are completely excluded. Nutrition is supplemented with low-fat dairy products (curdled milk, cottage cheese, whey, acidophilus). All kinds of vegetables and fruits are allowed. If their heat treatment is necessary, then choose the method of steaming, boiling, stewing. Twice a week, rabbit meat, chicken or turkey breast, quail meat, and guinea fowl are included in the diet. If bloodletting has been performed or the symptoms are pronounced, then the diet should be observed especially strictly.

bloodletting

In the treatment of polycythemia, bloodletting is indicated to remove a certain amount of red blood cells. Often, up to 400 ml of blood is taken from young people, and 100 ml from the elderly, and in the presence of cardiovascular diseases. Before the procedure, drugs are prescribed that reduce blood clotting. Manipulation is not carried out in the presence of blood clots.

Bloodletting, according to the ancient luminaries of medicine, promotes rejuvenation of the whole organism, activation of metabolic and regenerative processes. But the procedure requires a complete examination of the patient, is carried out under sterile conditions and only in a hospital or clinic. Laboratory diagnostics is required before and after the manipulation. Monitoring a person's condition is a prerequisite for medical supervision.

Transfusion of blood components

To restore the volume of circulating blood and replenish its formed elements, a transfusion of erythrocyte or platelet mass is prescribed. The procedure is carried out in three stages. They take into account the blood type, Rh factor of the patient, select a suitable portion, check it. A test is carried out for individual compatibility, a biological test.

Red blood cell transfusion is carried out in severe conditions of the patient, such as a severe stage of anemia and coma. The latter occurs with a sharp decrease in the number of red blood cells and insufficient enrichment of the brain with oxygen. A severe form of anemia occurs when the hemoglobin level drops below 70 g / l, given that the norm is 120-160 g / l. The platelet mass is transfused with a critical decrease in platelets in the blood (the norm is 180-420 * 109 / l), frequent and prolonged bleeding.

polycythemia - polycythemia

True polycythemia - what is it? Symptoms.

polycythemia - polycythemia

polycythemia - polycythemia

ethnoscience

Treatment with folk remedies does not always give a positive result, but, on the contrary, allows the disease to go into a latent form. It is best to use recipes "from the people" in combination with drug treatment, after consulting with your doctor in advance. It is necessary to take into account the individual characteristics of the organism, whether there are any allergies to certain herbal preparations. There are many folk methods that contribute to blood thinning in polycythemia.

Phytotherapy does not give a quick result, so the course of treatment can be several months. It is recommended to use herbal preparations for the prevention of the disease and during periods of its retreat. When treating, a sense of proportion is important, otherwise severe bleeding can be caused. First you need to balance your diet, add seafood, eat plenty of almonds and walnuts, limit foods containing vitamin K, drink clean water.

Recipe #1

If there is a diagnosis of "Relative (secondary) polycythemia, then you need to take cranberries (1-1.2 tablespoons), pour boiling water (200-250 ml), let it brew under a closed lid for thirty minutes. Use instead of tea with sugar or flower honey. Do not get carried away, the number of servings per day should be minimized. The course of treatment is one month.

These berries perfectly lower blood viscosity. From them you can make compotes, juices, fruit drinks. Contraindications are gastritis and stomach ulcers, because cranberries contain a large amount of acids. This recipe is especially useful if bloodletting has been performed.

Recipe #2

Flaxseed oil normalizes the metabolism of fats in the body, reduces the risk of atherosclerosis, blood clots, so the disease can be treated more effectively. As a result: the likelihood of developing myocardial infarction and coronary heart disease is reduced. The oil in its composition contains polyunsaturated fatty acids, which are indispensable in our body and must come from the external environment. Based on them, many pharmacological preparations have been created, and mother nature gives us valuable substances in their natural form.

Flaxseeds should be applied in a tablespoon in the morning before meals. For any reason, you can use it after meals. To achieve the effect, you should drink every day for a course of three weeks. Contraindications: gallstone disease, diarrhea.

Recipe #3

Horse chestnut is a good natural anticoagulant, and this property is very important to remove the main manifestation of the disease. The beneficial compound is found in all parts of the plant. The drug can be prepared in several ways. For alcohol tincture, you need to combine 50 grams of the peel of the fetus with 350 ml of vodka. Let it brew without access to light for 14-16 days. After taking three times a day. At one time, just 30 drops of infusion is enough.

Chopped chestnut leaves are also suitable, which will require 1 tbsp. spoon. Fill with boiling water (200 ml), wrap and leave for at least 12 hours. You need to use an infusion to thin the blood in the evening, half a glass after a meal. You can make a decoction: a glass of water needs a tablespoon of medicinal raw materials. Boil over low heat for 30 minutes, then take as infusion.

Recipe number 4

Herbal tea. A pleasant tea drinking will also turn into a healthy one, and the components can be varied to taste. The most common foods include: ginger, lemon, cranberries, motherwort and knotweed. Add a slice of lemon and a little grated ginger to your drink - this will give not only a rich, spicy taste, but also health.

For the next tea recipe you need: motherwort (3), cudweed, knotweed and Ivan tea (1 part each), mint (1). In total, you need to take 1 tsp. of this collection, pour a glass of water and simmer for 15 minutes, it is better to do this in a water bath. Then let it brew for 30 minutes. Use instead of tea for 12 hours a day, adding lemon, ginger or honey.

The disease occurs predominantly in the elderly (average age of onset is approximately 60 years), but is also diagnosed in young people and children. For younger patients, a more severe course of the disease is characteristic. Men are slightly more prone to polycythemia vera than women, but young patients are characterized by an inverse proportion.

Causes and risk factors

The causes contributing to the occurrence of true polycythemia have not been finally established. Pathology can be both hereditary and acquired. Found a family predisposition to the disease. In patients with true polycythemia, gene mutations are detected that are inherited in an autosomal recessive manner.

Risk factors include:

  • impact on the body of toxic substances;
  • ionizing radiation;
  • x-ray exposure;
  • extensive burns;
  • long-term use of a number of drugs (gold salts, etc.);
  • advanced forms of tuberculosis;
  • distress;
  • viral diseases;
  • tumor neoplasms;
  • smoking;
  • endocrine disorders caused by tumors of the adrenal glands;
  • heart defects;
  • diseases of the liver and / or kidneys;
  • extensive surgical interventions.

Forms of the disease

True polycythemia is of two types:

  • primary (not a consequence of other pathologies);
  • secondary (develops against the background of other diseases).

Stages of the disease

There are three stages in the clinical picture of polycythemia vera:

  1. Initial (malosymptomatic) - clinical manifestations are insignificant, duration is about 5 years.
  2. The erythremic (expanded) stage lasting 10–20 years, in turn, is divided into substages: IIA - myeloid metaplasia of the spleen is absent; IIB - the presence of myeloid metaplasia of the spleen;
  3. Stage of posterythremic myeloid metaplasia (anemic) with or without myelofibrosis; capable of developing into chronic or acute leukemia.

Symptoms

Polycythemia vera is characterized by a long asymptomatic course. The clinical picture is associated with an increased production of red blood cells in the bone marrow, which is often accompanied by an increase in the number of other cellular elements in the blood. An increase in platelet count leads to vascular thrombosis, which can cause strokes, myocardial infarction, transient ischemic attacks, etc.

In the later stages of the disease, there may be:

  • skin itching, aggravated by exposure to water;
  • bouts of pressing pain behind the sternum during physical exertion;
  • weakness, increased fatigue;
  • memory disorder;
  • headaches, dizziness;
  • erythrocyanosis;
  • eye redness;
  • visual impairment;
  • increased blood pressure;
  • spontaneous bleeding, ecchymosis, gastrointestinal bleeding;
  • varicose veins (especially neck veins);
  • short-term intense pain in the fingertips;
  • stomach ulcer and / or duodenal ulcer;
  • joint pain;
  • heart failure.

Diagnostics

The diagnosis of polycythemia vera is established on the basis of data obtained during the examination:

  • collection of anamnesis;
  • objective examination;
  • general and biochemical blood test;
  • general urine analysis;
  • trepanobiopsy followed by histological analysis of the biopsy;
  • ultrasound examination;
  • computed or magnetic resonance imaging;
  • molecular genetic analysis.

Diagnostic criteria for polycythemia vera:

Differential diagnosis is required with absolute and relative (false) erythrocytosis, neoplasms, hepatic vein thrombosis.

Treatment

Treatment of polycythemia vera is aimed primarily at preventing the development of leukemia, as well as the prevention and / or therapy of thrombohemorrhagic complications. Symptomatic therapy is carried out in order to improve the patient's quality of life.

To reduce blood viscosity in hyperviscosity syndrome, a course of phlebotomy (exfusion, bloodletting) is performed. However, with initially high thrombocytosis, phlebotomy can contribute to the occurrence of thrombotic complications. Myelosuppressive therapy is indicated for patients who do not tolerate bloodletting, as well as in childhood and adolescence.

Interferon preparations are prescribed for a long course (2-3 months) to reduce myeloproliferation, thrombocythemia, and also to prevent the development of vascular complications.

With the help of hardware methods of therapy (erythrocytapheresis, etc.), excess blood cells are removed. In order to prevent thrombosis, anticoagulants are prescribed. To reduce the manifestations of itching, antihistamines are used. In addition, patients are advised to adhere to a dairy-vegetarian diet and limit physical activity.

With a pronounced increase in the size of the spleen (hypersplenism), splenectomy is indicated for patients.

Possible complications and consequences

Polycythemia vera can be complicated by:

  • myelofibrosis;
  • spleen infarction;
  • anemia;
  • nephrosclerosis;
  • cholelithiasis and / or urolithiasis;
  • gout;
  • myocardial infarction;
  • ischemic stroke;
  • cirrhosis of the liver;
  • pulmonary embolism;
  • acute or chronic leukemia.

Forecast

With timely diagnosis and treatment, survival exceeds 10 years. Without adequate therapy, 50% of patients die within 1-1.5 years from the moment of diagnosis.

Prevention

Due to the fact that the exact causes of the disease are unclear, effective methods for the prevention of polycythemia vera have not yet been developed.

Video from YouTube on the topic of the article:

Education: "First Kyiv Medical College", specialty "Laboratory diagnostics".

The information is generalized and is provided for informational purposes only. Seek medical attention at the first sign of illness. Self-medication is dangerous to health!

In order to say even the shortest and simplest words, we use 72 muscles.

Human bones are four times stronger than concrete.

Millions of bacteria are born, live and die in our intestines. They can only be seen at high magnification, but if they were brought together, they would fit in an ordinary coffee cup.

Dentists have appeared relatively recently. Back in the 19th century, pulling out diseased teeth was part of the duties of an ordinary hairdresser.

Scientists from the University of Oxford conducted a series of studies in which they came to the conclusion that vegetarianism can be harmful to the human brain, as it leads to a decrease in its mass. Therefore, scientists recommend not to completely exclude fish and meat from your diet.

An educated person is less prone to brain diseases. Intellectual activity contributes to the formation of additional tissue that compensates for the diseased.

Human blood “runs” through the vessels under enormous pressure and, if their integrity is violated, is capable of firing at a distance of up to 10 meters.

The average life expectancy of left-handers is less than that of right-handers.

According to WHO studies, a daily half-hour conversation on a mobile phone increases the likelihood of developing a brain tumor by 40%.

Falling off a donkey is more likely to break your neck than falling off a horse. Just don't try to disprove this claim.

The first vibrator was invented in the 19th century. He worked on a steam engine and was intended to treat female hysteria.

A job that a person does not like is much more harmful to his psyche than no job at all.

Even if a person's heart does not beat, he can still live for a long period of time, as the Norwegian fisherman Jan Revsdal demonstrated to us. His "motor" stopped for 4 hours after the fisherman got lost and fell asleep in the snow.

In addition to people, only one living creature on planet Earth suffers from prostatitis - dogs. These are really our most faithful friends.

Our kidneys are able to purify three liters of blood in one minute.

Prostatitis is an inflammatory process in the prostate gland. This is one of the most common diseases of the genitourinary system in men. How.

Preventive and dietary nutrition for hematological diseases (blood diseases)

There are diseases, such as, for example, a tumor of the intestine, in which it is believed that from 60 to 80% of the occurrence of a tumor depends on the type of food. In the case of blood diseases, there is no such clear relationship. However, it is safe to say that if a person constantly abuses fatty or carbohydrate foods, if he is used to eating at night, then he is much more likely to develop oncological and hematological diseases than a person who eats rationally.

Why can't you eat at night? The fact is that our body should have a rest time for the digestive system. Of course, many men, when they leave for work early in the morning, and return home only at 8 or 9 pm, say: “But when do I have to eat?” You can eat like that, but you need to know that you have to pay for everything in life. Therefore, either in some way you need to organize yourself a proper, competent diet during the day, or you will have to pay with your health for neglecting the requirements of the body.

Of course, a person should "eat breakfast himself, share lunch with a friend, and give dinner to the enemy." If you leave for work at 6-7 in the morning and cannot eat on time, then it is advisable that you still be able to have breakfast at work before 10 am.

In addition, after 45 years it is advisable to consume less butter and other animal fats by increasing olive, linseed or at least sunflower oil in the diet - after all, vegetable oils are rich in unsaturated fatty acids that help our body in all situations.

It’s best not to overeat and try to predominantly consume “real” food, i.e. plant foods and dairy products. It's good to know that dairy protein is much easier to digest than meat protein. It is not for nothing that there are people who eat only vegetables, fruits, dairy products, sometimes fish, but do not eat meat at all, and they, as a rule, feel much better and get sick less.

You also need to understand that a person must have a chair in the morning, otherwise the liquid from the feces begins to be absorbed into the lymph and blood flow and self-poisoning of the body occurs. If this happens constantly, the tendency to oncological and hematological diseases increases. Therefore, half of the foods consumed should be of plant origin, whether it be vegetables or fruits, since the fiber of plant foods contributes to the proper formation of feces, preventing it from being excessively compacted.

From plant products, it is better to give preference to those vegetables, fruits and berries that grow in your area. It is advisable to stick to seasonal nutrition: spring time begins, the first grass appears, lettuce, dill, parsley - buy these products. In winter, sauerkraut is very useful.

Diet for polycythemia

With polycythemia, it is not recommended to eat food that can lead to an increase in the already large number of blood cells. Such food includes all foods that are red, in particular, with thrombocythemia, we recommend not to abuse meat, chocolate, red berries and fruits, red vegetables - all that involuntarily leads to an increase in hemoglobin and red blood cells. On the other hand, people who have reduced hemoglobin just need all of the above products. To increase the level of hemoglobin and erythrocytes, cereals from buckwheat and oatmeal (or "Hercules") are useful, cranberries and lingonberries increase the level of erythrocytes well.

Description of the disease Children with abnormal clotting experience more prolonged, excessive or sudden bleeding than healthy children. For example, the socket of an extracted tooth in such children very often bleeds for four or even six hours after the doctor removed the tooth. The joint can fill with blood after a slight. ⇒

Nutrition for leukemia

blood diseases, leukemia, nutrition All people suffering from leukemia are strongly recommended foods high in animal protein (up to 115 grams), vitamins and fat restriction (up to 35 grams). In the daily diet should be fresh vegetables, berries, fruits and fresh. ⇒

Analysis to detect hidden bleeding from the intestines

Recently, a new test has appeared to detect hidden bleeding from the intestine. In what cases is it carried out? Maria, 42, Moscow The research is aimed at early detection and prevention of colorectal cancer. This is a quantitative immunochemical method that helps to detect hidden bleeding from the lower gastrointestinal tract. Colorectal. ⇒

How to support the heart

The husband at work began to earn not only money, but also diseases. He came home excited, nervous, could not fall asleep for a long time. Then things got even worse - my heart began to ache. Suddenly, the spouse could have tachycardia, tingling. I managed to persuade him to go to the doctor only through. ⇒

Add a comment Cancel reply

Comparison of the efficacy and safety of drugs for weight loss

How not to make a mistake with the choice of means for weight loss

How to lose weight fast in 3 days without dieting?

What method of treatment of osteochondrosis to choose?

Treatment of bleeding gums

Treatment of periodontitis

Protruding ears in humans: types of deformity and surgical elimination

Medicine worthy of attention

Drugs for unwanted pregnancy

Causes of scoliosis in childhood

Swim first, then walk

Disorders in the blood coagulation system in a child

First pregnancy

5 Day Ovulation: Early Pregnancy Symptoms

Joint birth in Germany - pros and cons

Garlic is a universal medicine

Benefits of Lemon Balm and How to Use It

Inflammation of the gums, treatment with folk remedies

  • Online calculator for calculating the ideal weight using advanced formulas, taking into account age and physique - rating:
  • How to calculate your normal weight? - rating:
  • Points where you can put medical leeches - rating:
  • Is it possible to stop the destruction of the liver in hepatitis C? - rating:
  • The child has a stomachache. What to do? - rating:
  • Found a cure for hepatitis C! - rating:

Medical Academy for the Whole Family © 2018. All rights reserved.

The essence and differences between true and secondary polycythemia

Polycythemia is a condition of the body in which the production of red blood cells increases pathologically. Often this is associated with an increase in total blood volume and spleen, the number of leukocytes and platelets is also increased. There is a gender predisposition: men are less resistant to the disease, they get sick several times more often than the female half of the population. The most vulnerable period is the middle and older age, but polycythemia is increasingly being recorded in newborns.

Classification and symptoms

The classification of polycythemia is quite detailed. It is customary to single out not only the forms, but also the stages of the disease. This allows you to more accurately prescribe a course of therapy and rehabilitation. There are several forms of the disease:

  • polycythemia vera (primary, Wakez's disease). The erythroblastic germ in the bone marrow undergoes hyperplastic changes.
  • relative polycythemia (secondary, Gaisbeck's syndrome).

Additionally, polycythemia vera is divided into 3 stages:

  • primary. It develops at the onset of the disease, the duration of the course may exceed a five-year period. The manifestation of symptoms is mild or there is no symptomatic picture at all;
  • expanded. It may take up to 20 years. Occurs with and without damage to the spleen.
  • heavy. It is characterized by the formation of oncological tumors of any localization, but more often not only the spleen, blood, but also the liver suffers.

Secondary polycythemia (Gaisbeck's syndrome) can develop as a reaction to prolonged stress, poor working conditions and lack of proper rest. Such a course can pass with a minimum therapeutic course, but subject to the eradication of the root cause of the disease. True polycythemia may be false. It does not lead to a shift in the erythrocyte, leukocyte, platelet balance.

The reasons

The pathogenesis of the disease largely depends on the form of the disease. It has been noted that polycythemia vera develops due to gene mutations, hereditary predisposition to distortion of hematopoietic processes. Pathology occurs with prolonged and significant hypoxia. All oncological processes that have formed in the bone marrow can have a detrimental effect on the natural and well-formed mechanism for the production of red blood cells. Secondary polycythemia can develop as a result of:

  • previous obstructive pulmonary disease;
  • pulmonary hypertension;
  • long-term failure of the heart;
  • oxygen starvation of the kidneys, as the main organ of the excretory system;
  • unfavorable climate, especially in high mountains;
  • oncological process of any localization;
  • exposure to infectious agents;
  • some features of labor (miners, high-altitude workers, etc.);
  • unhealthy environmental conditions (living in megacities, near hazardous industries, near major highways);
  • malicious smoking;
  • belonging to a European nation. Blame the prevailing genotype.

Many of the causes of the symptoms of the disease have not been studied. In particular, neonatal polycythemia occurs as a result of a violation of placental activity. The baby takes over the disease from the mother, while still in her womb. The transmission mechanism of the disease is poorly understood by doctors. Today, measures are being worked out to prevent this method of transmission of the disease.

Symptomatic picture

The first signs (syndrome) of the disease usually do not cause suspicion. In the early stages, a paroxysmal headache, visual impairment, a person loses sleep, complains of dizziness, nausea. The fingertips, both on the upper and lower limbs, are cold. Their temperature is practically independent of the ambient temperature.

In the expanded stage, muscle pain appears. The joints "ache" almost around the clock. The general condition worsens, working capacity, stress resistance, the ability to memorize large amounts of new information fall. The skin becomes bruised, the gums begin to bleed, nosebleeds can occur, provoked by minimal trauma to the mucosa. The spleen and liver are often enlarged. These are non-specific symptoms, but there are also specific signs. These include:

  • severe itching. Moreover, itching increases after any skin contact with water. Sometimes, it becomes unbearable, which leads to neurotic states;
  • in the pads on the fingers there is a burning sensation of varying intensity;
  • the skin of the face becomes red with a bluish tint;
  • saphenous veins are visualized;
  • persistent essential hypertension often occurs;
  • lips, tongue, whites of the eyes are filled with blood;
  • general weakness continues to progress, it is characteristic of the disease;
  • the risk of thrombosis, heart attacks, strokes increases sharply;
  • liver tissue changes according to the type of cirrhosis, the organ increases in size.

In the last stage of the disease, when uncontrolled growth of tumors leads to a critical drop in body weight and general intoxication, the entire symptomatic picture is pronounced. Anemia increases, the risk of gastric, uterine, intestinal bleeding increases. In the absence of competent treatment, or in case of detection of the disease in a late stage, a fatal outcome occurs.

Polycythemia in newborns

Neonatal polycythemia manifests itself 6-7 days after birth. More often, symptoms appear in full-term and large twins. The skin is painful, even a slight touch causes the child to cry. A reddish color of the skin appears, itching, body weight drops. Primary symptoms (manifestations) are confirmed by laboratory tests: erythrocytes, leukocytes and platelets significantly exceed the age norm. The liver and spleen are enlarged.

Diagnostic measures and prognosis

As soon as the primary signs of the disease are suspected, a qualitative diagnosis is necessary. It begins with a detailed history taking. Find out a possible hereditary origin, which requires a "family" history. Further, laboratory tests are prescribed: a general analysis of blood and urine. If deviations from the norm are detected, then the blood is examined for biochemistry and the leukocyte formula is determined.

In order to confirm or refute the diagnosis, it is necessary to conduct a histology and morphology of bone marrow punctate. A cytological examination will also be required. The causes and degree of damage to the body during the disease will help to identify ultrasound of the internal organs and blood vessels, FGDS, echocardiography, etc. You may need to consult related specialists: urologist, pulmonologist, gynecologist. The hematologist resolves the issue of dispensary registration.

Forecast

The prognosis of the disease depends on many factors: the severity and malignancy of the pathology, the correct treatment, the age of the patient, the individual capabilities of the body, etc. Only 1% of polycythemia transforms into leukemia, and up to 15% of patients need cytostatics. But the disease is not prone to self-healing or long-term "spontaneous" remissions. Lifelong follow-up with a hematologist is required. In severe cases, disability is assigned, it is typical for many patients with a similar diagnosis. In order for the prognosis to become more favorable, it is necessary to identify the symptoms in time and take full courses of treatment, carefully following medical prescriptions.

Treatment of polycythemia

To prescribe the right method of therapy, it is necessary to diagnose the exact cause of the disease. With true polycythemia, it is necessary to influence neoplasms in the red bone marrow, with secondary (Gaisbeck's syndrome), the cause that caused the disease is eliminated. Therapy for polycythemia vera is a rather lengthy and complex process. Therefore, there must be adequate treatment that affects the tumor and prevents its metastasis. When choosing medicines, the age of the patient must be taken into account, not all drugs are equally good for young and elderly patients.

Medical treatment

Therapy is aimed at eliminating the symptoms caused by the disease. With high blood pressure, antihypertensive drugs (enalapril, captopril, coverex) are recommended. Antihistamines are used to relieve itching (Fenkarol, Suprastinex) and other body responses. Antiplatelet agents (Vixipin, aspirin-based drugs) are used to reduce blood clotting.

Anticoagulants are prescribed to reduce the risk of blood clots. This pharmacological group includes Heparin, Worfarin, Dalteparin and antiplatelet drugs. When bleeding, it is appropriate to prescribe hemostatic agents. These include Vikasol, Epsilon-aminocaproic acid, Hemofobrin. Taking diuretics is strictly prohibited, they thicken the blood even more and cause various complications.

The use of interferons and tranquilizers

Interferons are specific proteins produced by the immune system. They are aimed at combating pathogenic flora, destroying the DNA and RNA of pathogens. Modern pharmacologists have created artificial interferons with a similar property, because drugs in this group improve the prognosis and reduce the signs of blood disease. The most commonly shown application is:

Separately, I would like to say about tranquilizers in the treatment of Wakez's disease. Tranquilizers are drugs that have a calming effect. They are indicated if complex diagnostics, bloodletting is needed. Or there is itching, there is a fear of chemotherapy. In such cases, the use of Lorazepam, Ozepam, Medazepam (rudotel), Alprazolam (Xanax), Chlordiazepoxide (Elenium), Gidazepam, Diazepam (Valium), Seduxen, Relanium, Sibazon is indicated.

Chemotherapy

In cases where the disease progresses, a serious condition of the patient is observed, complications from the internal organs and blood vessels join, then chemotherapy is recommended. The drugs used in this case have a detrimental effect on tumor cells. Apply Hydroxyurea, Anagrelide and Bisulfan. The treatment process should be complex, therefore, drug therapy and prevention are used.

Non-drug therapy: briefly

This type of treatment is used in combination with medications. Only an integrated approach will provide a positive prognosis for recovery. You can not neglect any opportunity to stop the progression of the disease, otherwise it will be very difficult to achieve remission. Here are the most useful methods.

diet therapy

Meat and fish are left in the diet at the very least. But their fatty varieties are completely excluded. Nutrition is supplemented with low-fat dairy products (curdled milk, cottage cheese, whey, acidophilus). All kinds of vegetables and fruits are allowed. If their heat treatment is necessary, then choose the method of steaming, boiling, stewing. Twice a week, rabbit meat, chicken or turkey breast, quail meat, and guinea fowl are included in the diet. If bloodletting has been performed or the symptoms are pronounced, then the diet should be observed especially strictly.

bloodletting

In the treatment of polycythemia, bloodletting is indicated to remove a certain amount of red blood cells. Often, up to 400 ml of blood is taken from young people, and 100 ml from the elderly, and in the presence of cardiovascular diseases. Before the procedure, drugs are prescribed that reduce blood clotting. Manipulation is not carried out in the presence of blood clots.

Bloodletting, according to the ancient luminaries of medicine, promotes rejuvenation of the whole organism, activation of metabolic and regenerative processes. But the procedure requires a complete examination of the patient, is carried out under sterile conditions and only in a hospital or clinic. Laboratory diagnostics is required before and after the manipulation. Monitoring a person's condition is a prerequisite for medical supervision.

Transfusion of blood components

To restore the volume of circulating blood and replenish its formed elements, a transfusion of erythrocyte or platelet mass is prescribed. The procedure is carried out in three stages. They take into account the blood type, Rh factor of the patient, select a suitable portion, check it. A test is carried out for individual compatibility, a biological test.

Red blood cell transfusion is carried out in severe conditions of the patient, such as a severe stage of anemia and coma. The latter occurs with a sharp decrease in the number of red blood cells and insufficient enrichment of the brain with oxygen. A severe form of anemia occurs when the hemoglobin level drops below 70 g / l, given that the norm is g / l. The platelet mass is transfused with a critical decrease in platelets in the blood (norm * 109 / l), frequent and prolonged bleeding.

What is true polycythemia. What are the symptoms

ethnoscience

Treatment with folk remedies does not always give a positive result, but, on the contrary, allows the disease to go into a latent form. It is best to use recipes "from the people" in combination with drug treatment, after consulting with your doctor in advance. It is necessary to take into account the individual characteristics of the organism, whether there are any allergies to certain herbal preparations. There are many folk methods that contribute to blood thinning in polycythemia.

Phytotherapy does not give a quick result, so the course of treatment can be several months. It is recommended to use herbal preparations for the prevention of the disease and during periods of its retreat. When treating, a sense of proportion is important, otherwise severe bleeding can be caused. First you need to balance your diet, add seafood, eat plenty of almonds and walnuts, limit foods containing vitamin K, drink clean water.

Recipe #1

If there is a diagnosis of "Relative (secondary) polycythemia, then you need to take cranberries (1-1.2 tablespoons), pour boiling water (ml), let it brew under a closed lid for thirty minutes. Use instead of tea with sugar or flower honey. Do not get carried away, the number of servings per day should be minimized. The course of treatment is one month.

These berries perfectly lower blood viscosity. From them you can make compotes, juices, fruit drinks. Contraindications are gastritis and stomach ulcers, because cranberries contain a large amount of acids. This recipe is especially useful if bloodletting has been performed.

Recipe #2

Flaxseed oil normalizes the metabolism of fats in the body, reduces the risk of atherosclerosis, blood clots, so the disease can be treated more effectively. As a result: the likelihood of developing myocardial infarction and coronary heart disease is reduced. The oil in its composition contains polyunsaturated fatty acids, which are indispensable in our body and must come from the external environment. Based on them, many pharmacological preparations have been created, and mother nature gives us valuable substances in their natural form.

Flaxseeds should be applied in a tablespoon in the morning before meals. For any reason, you can use it after meals. To achieve the effect, you should drink every day for a course of three weeks. Contraindications: gallstone disease, diarrhea.

Recipe #3

Horse chestnut is a good natural anticoagulant, and this property is very important to remove the main manifestation of the disease. The beneficial compound is found in all parts of the plant. The drug can be prepared in several ways. For alcohol tincture, you need to combine 50 grams of the peel of the fetus with 350 ml of vodka. Let it brew without access to light for 14-16 days. After taking three times a day. At one time, just 30 drops of infusion is enough.

Chopped chestnut leaves are also suitable, which will require 1 tbsp. spoon. Fill with boiling water (200 ml), wrap and leave for at least 12 hours. You need to use an infusion to thin the blood in the evening, half a glass after a meal. You can make a decoction: a glass of water needs a tablespoon of medicinal raw materials. Boil over low heat for 30 minutes, then take as infusion.

Recipe number 4

Herbal tea. A pleasant tea drinking will also turn into a healthy one, and the components can be varied to taste. The most common foods include: ginger, lemon, cranberries, motherwort and knotweed. Add a slice of lemon and a little grated ginger to your drink - this will give not only a rich, spicy taste, but also health.

For the next tea recipe you need: motherwort (3), cudweed, knotweed and Ivan tea (1 part each), mint (1). In total, you need to take 1 tsp. of this collection, pour a glass of water and simmer for 15 minutes, it is better to do this in a water bath. Then let it brew for 30 minutes. Use instead of tea for 12 hours a day, adding lemon, ginger or honey.

All information on the site is provided for informational purposes only. Be sure to consult your doctor before using any recommendations.

Is polycythemia vera treated with folk remedies?

Chronic pathology of the blood of a tumor nature, characterized by a constant and significant increase in red blood cells per unit volume of blood, as well as splenomegaly and redness of the skin and mucous membranes, is called polycythemia. Many people ask the question: “Is it possible to treat polycythemia vera with folk remedies?”.

It is important to understand that the disease is dangerous, and only a qualified specialist can prescribe the use of a particular method. Therapy with herbal ingredients is possible, but only as an auxiliary.

What provokes the development of true polycythemia and what are its symptoms?

For the first time, the symptoms of the disease were described by the therapist Henri Louis Vaquez in 1892. Polycythemia is called a disease of the second half of life. And there is an explanation for this. Often the disease is diagnosed in people after 40 years. It is very rare in the younger generation. Men are more often affected by the development of the disease.

The occurrence of the disease may be due to genetic predisposition, exposure to ionizing radiation and toxic substances, the presence of hydronephrosis, cysts and tumors of the kidneys, chronic lung pathologies, and smoking.

Polycythemia is usually characterized by:

  • redness of the skin and mucous membranes;
  • painful sensations in the fingers of the upper and lower extremities;
  • headaches;
  • enlargement of the liver and spleen;
  • itching of the skin;
  • joint pain;
  • malfunctions of the digestive tract;
  • thinning and increased fragility of hair;
  • dry skin and mucous membranes;
  • malaise;
  • a feeling of lack of air;
  • thrombosis.

The worst thing is that pathology does not make itself felt immediately, a person can live with it and at the same time not even be aware of its presence. That is why, even if symptoms such as malaise or headache appear, which we often attribute to fatigue, seek the help of a doctor.

Folk remedies in the fight against the disease

In parallel with drug treatment and other methods of therapy, you can use recipes from folk remedies: plants that help thin the blood, normalize the general condition, strengthen the immune system and prevent the formation of blood clots. However, it is not recommended to use it without the knowledge of a doctor. Self-medication can cause aggravation of symptoms!

1. Cranberries in the fight against pathology. Brew 30 grams of fresh or dried cranberries in two hundred milliliters of boiling water. Remove the container in heat for half an hour. Drink a drink instead of tea. You can add honey or sugar to improve the taste.

2. The use of sweet clover. Steam 10 grams of dried ground part of the plant with boiled water - a glass. Leave the composition to infuse. Drink ½ cup of the drink twice a day. The duration of the therapeutic course is a month.

3. Application of healing tincture. Grind the peel of horse chestnut fruits and pour 50 grams of vodka - 500 ml. Remove the tightly closed container in a dark place for half a month. Use a spoonful of filtered drug, diluted with warm water - in a quarter cup three times a day, before meals. The duration of the therapeutic course is 21 days.

4. Treatment of polycythemia with garlic tincture. Take a few heads of garlic, chop with a meat grinder. Pour raw materials into a glass bottle and fill with vodka. Leave to infuse in a cool dark place for half a month. Next, combine the filtered liquid in equal proportions with lemon juice and honey. Mix well and use a spoonful of the drug once a day, before going to bed.

5. Application of miraculous infusion. Mix in equal proportions mountain arnica with wormwood, sweet clover and meadowsweet. Brew 20 grams of raw materials in three hundred milliliters of freshly boiled water. Leave to brew overnight. Drink 100 ml of strained drink three times a day. The course of therapy is thirty days.

6. Treatment of the disease with a ginger drink. Grind the fresh rhizome of the plant, and then mix with green tea - a spoon and cinnamon - a pinch. Brew raw materials with half a liter of boiling water. Keep warm for an hour. Drink ¼ cup of the drink four times a day.

7. Therapy of the disease with orange juice. Drink 100 ml of freshly squeezed juice daily.

8. Application of an effective tincture. Pour 50 grams of dry, finely chopped ginkgo biloba leaves with alcohol - half a liter. Put the container in a dark place for three weeks. Take 10 drops of the filtered potion before each meal.

10. Treatment of pathology with carrot juice. It is advised to drink 300 ml of freshly squeezed carrot juice daily.

Take your medicines regularly, but do not forget about nutrition

Along with the use of medicinal and herbal preparations from folk remedies, it is necessary to monitor nutrition. First of all, enrich the diet with foods that thin the blood. It is recommended to use more:

  • seafood,
  • fish,
  • seaweed,
  • nuts,
  • bell pepper,
  • fresh garlic and onion
  • tomatoes,
  • pumpkins,
  • zucchini,
  • celery,
  • cucumbers,
  • linseed oil
  • string beans,
  • ginger,
  • melon,
  • cherries,
  • strawberries,
  • blueberries,
  • citrus,
  • sprouted wheat,
  • bitter chocolate,
  • dairy products - kefir, milk, curdled milk, yogurt.

Minimize meat consumption - no more than twice a week. It is preferable to buy low-fat varieties. Pay special attention to the drinking regimen. A person needs to drink two liters of water per day. Information will be useful: "Healing energy of melt water."

Prevention of polycythemia

In order to prevent the development of this disease, it is necessary to lead an active and healthy lifestyle, give up addictions, in particular smoking, and regularly take a blood test and undergo a medical examination. In addition, treat on time pathologies that can provoke the development of polycythemia.

All rights reserved. Copying of material is only possible

Today we will talk about such a blood disease as polycythemia vera. This disease is a pathology in which there is an increased number of red blood cells in the circulating blood. Polycythemia carries a great, sometimes irreversible danger to human life and health, so it is important to recognize the disease by its first signs for timely medical care and competent treatment. Typically, this syndrome is characteristic of people over the age of 50, and is more often diagnosed in males. Let us consider in more detail the disease in all its aspects: etiology, types, diagnosis and main methods of curing polycythemia.

General information about the disease

In modern medicine, polycythemia has several names, for example, it is also sometimes called erythrocytosis. Pathology belongs to the section of chronic leukemia and represents an active increase in the concentration of erythrocytes, leukocytes and platelets in the blood, most often specialists refer this disease to a rare type of leukemia. Medical statistics say that true polycythemia is diagnosed annually only in 5 cases per 1 million patients, usually the development of pathology is typical for older men (from 50 to 65 years).

The most dangerous complications of the disease include the risk of developing thrombosis and hemorrhagic strokes, as well as the transition of polycythemia to the acute stage of myeloid leukemia or to the chronic stage of myeloid leukemia. This disease is characterized by a number of reasons, which we will consider below. All causes of erythremia are divided into two types: primary and secondary.

Causes of the disease

In modern medicine, the root causes of this pathology include the following:

  • genetic predisposition to increased production of red blood cells;
  • failures at the genetic level;
  • oncological diseases of the bone marrow;
  • oxygen deprivation also affects the increased production of blood cells.

Most often, erythremia has a tumor factor, characterized by damage to stem cells produced in the red bone marrow. The result of the destruction of these cells is an increase in the level of erythrocytes, which directly leads to disruption of the whole organism. The disease is malignant, difficult to diagnose and treated for a long time, and not always with a positive effect, complex therapy is due to the fact that no treatment methods can affect a stem cell that has undergone a mutation, which has a high ability to divide. True polycythemia is characterized by the presence of plethora, this is due to the fact that the concentration of red blood cells is increased in the vascular bed.

In patients with polycythemia, purplish-red skin is observed, often patients complain of itching.

The secondary causes of the disease experts include factors such as:

  • pathology of the lungs of an obstructive nature;
  • heart failure in a chronic form;
  • there is not enough oxygen supply to the kidneys;
  • a sharp change in climate, and the development of this syndrome is typical for the population living in high mountainous areas;
  • various infections leading to high intoxication of the body;
  • harmful working conditions, especially for work carried out at height;
  • the disease also affects people living in ecologically polluted areas, or in close proximity to industries;
  • excessive smoking;
  • experts have revealed that a high risk of developing polycythemia is characteristic of people with Jewish roots, this is due to the genetic feature of the function of the red bone marrow;
  • sleep apnea;
  • hypoventilation syndromes leading to polycythemia.

All these factors lead to the fact that hemoglobin is endowed with the ability to actively attach oxygen to itself, while there is practically no return to the tissues of internal organs, which, accordingly, leads to the active production of red blood cells.

It is worth noting that some cancers can also provoke the development of erythremia, for example, tumors of the following organs affect the production of red blood cells:

  • liver;
  • kidneys;
  • adrenal glands;
  • uterus.

Some kidney cysts and obstruction of this organ can increase the secretion of blood cells, leading to the development of polycythemia. Sometimes polycythemia occurs in newborns, this disease is transmitted through the maternal placenta, there is an insufficient supply of oxygen to the fetus, as a result of which pathology develops. Next, consider the course of polycythemia, its symptoms and treatment, what are the complications of polycythemia?

Symptoms of polycythemia

This disease is dangerous because polycythemia vera at the initial stage is almost asymptomatic, the patient does not have any complaints about deteriorating health. Most often, pathology is detected during a blood test, sometimes the first “rings” of polycythemia are associated with colds or simply with a general decrease in efficiency in the elderly.

The main signs of erythrocytosis include:

  • a sharp drop in visual acuity;
  • frequent migraines;
  • dizziness;
  • noise in ears;
  • sleep problems;
  • "icy" fingers.

When the pathology enters the advanced stage, then with polycythemia, the following can be observed:

  • muscle and bone pain;
  • ultrasound is often diagnosed with an enlarged spleen, or a change in the contours of the liver;
  • bleeding gums;
  • for example, when a tooth is removed, the blood may not stop for a long time;
  • patients often find new bruises on their bodies, the origin of which they cannot explain.

Doctors also identify specific symptoms of this disease:

  • severe skin itching, which increases after taking water procedures;
  • burning sensation of the fingertips;
  • the appearance of vascular "asterisks";
  • the skin of the face, neck and chest may acquire a purple-red tint;
  • lips and tongue, on the contrary, may have a bluish tint;
  • the whites of the eyes are prone to redness;
  • the patient constantly feels weak.

If we talk about a disease that affects newborns, then polycythemia develops a few days after birth. Most often, pathology is diagnosed in twins, the main signs include:

  • the baby's skin turns red;
  • when touching the skin, the child experiences discomfort, so he starts to cry;
  • the baby is born small;
  • a blood test reveals an increased level of leukocytes, platelets and erythrocytes;
  • on ultrasound, changes in the size of the liver and spleen are observed.

It should be noted that if polycythemia is not diagnosed in time, the development of the disease can be missed, and the lack of therapy can lead to the death of the newborn.

Diagnosis of the disease

As mentioned above, most often true polycythemia is detected during prophylactic blood donation for analysis. Specialists diagnose erythrocytosis if, in a blood test, the indicators show a level above the norm:

  • hemoglobin level increased to 240 g/l;
  • the level of erythrocytes is increased to 7.5x10 12 /l;
  • the level of leukocytes is increased to 12x10 9 /l;
  • platelet level increased to 400x10 9 /l.

To study the function of the red bone marrow, a trepanobiopsy procedure is used, because it is the violation of stem cell production that provokes the development of polycythemia. To exclude other diseases, specialists can use studies such as ultrasound, urinalysis, FGDS, ultrasound, and so on. Also, the patient is prescribed consultations with narrow specialists: a neurologist, a cardiologist, a urologist, etc. If a patient is diagnosed with polycythemia, what is the treatment for this disease, consider the main methods.

Treatment of erythrocytosis

This disease belongs to those types of pathology that are treated with myelosuppressive drugs. Also, the treatment of polycythemia vera is carried out by methods of bloodletting, this type of therapy can be prescribed to patients who have not reached the age of 45. The essence of the procedure is that up to 500 ml of blood is taken from the patient per day, phlebotomy is also performed for elderly people with polycythemia, only blood is taken no more than 250 ml per day.

If a patient with this disease has severe itching and hypermetabolic syndrome, then specialists prescribe a myelosuppressive method of treating polycythemia vera. It includes the following drugs:

  • radioactive phosphorus;
  • anagrelide;
  • interferon;
  • hydroxyurea.

In the case of remission with polycythemia, the patient is prescribed repeated blood tests no more than once every 14 days, then the study is carried out once a month. When the level of erythrocytes returns to normal, the drugs begin to be gradually canceled, there is an alternation of drug therapy with rest from drugs, while the course of the disease is strictly observed. But it is worth noting that the use of myelosuppressive drugs in polycythemia can lead to the development of leukemia, therefore, specialists prescribe them after lengthy detailed studies. Sometimes there are side effects such as skin ulcers, disruption of the gastrointestinal tract, fever, if this happens, then the medications are immediately canceled.

Also, the patient should take Aspirin daily to reduce the risk of developing thrombosis, which often complicates the course of this disease.

A patient with polycythemia is also shown a procedure such as erythrocytophoresis, which consists in the fact that the device pumps out blood from the patient, while simultaneously removing excess red blood cells from it. After that, in order to restore the previous volume, the patient is infused with saline, this procedure is a modern type of bloodletting, but it is carried out no more than once every 2-3 years. Treatment of polycythemia will not insure the patient against possible complications that may develop against the background of this pathology.

Complications of polycythemia

Experts note the following complications that accompany the development of true polycythemia:

  • urine can acquire a sharp and unpleasant odor;
  • often patients with polycythemia suffer from gout;
  • with polycythemia, kidney stones can form;
  • renal colic pass into the chronic stage;
  • often erythrocytosis accompanies a stomach or duodenal ulcer;
  • impaired circulatory function can lead to the formation of skin ulcers;
  • often this disease provokes thrombosis;
  • bleeding gums, frequent nosebleeds.

Preventive measures

The development of a disease such as polycythemia can be prevented, it is necessary to adhere to the following preventive measures:

  • completely abandon bad habits, especially cigarette smoking, it is nicotine that harms the body and provokes this disease;
  • if the area is unfavorable for living, then it is better to change the place of residence;
  • the same applies to work;
  • regularly take preventive blood tests, which can show the presence of polycythemia in the patient;
  • it is necessary to take a responsible attitude to your diet, it is better to limit the consumption of meat, include in your diet those foods that stimulate the function of hematopoiesis, give preference to sour-milk and vegetable products.

Remember that timely diagnosis and competent treatment of polycythemia can prevent the development of complications in this disease, but, unfortunately, with this disease there is no guarantee of a complete cure.

In contact with

Similar posts