Erythremia in children symptoms. Improved blood flow. Symptoms of the initial stage

Erythremia is a persistent progressive increase in the number of red blood cells as a result of deep violation regulation of blood formation, which is the main clinical expression of the disease.

Systemic disease hematopoietic organs, close to tumors (as well as leukemia) with uncontrollable growth of erythroblastic tissue bone marrow(and spleen) and a corresponding progressive increase in the number of erythrocytes in the peripheral blood, thrombosis of the vessels of the brain, intestines, etc. To a lesser extent, with erythremia, the formation of leukocytes and plates in the bone marrow is increased.

Causes of erythremia

Persons of 40-50 years of age are mostly affected. The cause of the disease is mostly unknown. In some cases, there is exposure to infections (in case of tuberculosis of the spleen), poisons (in the experiment, a state similar to erythremia was caused by the introduction of cobalt) or brain damage, disease-causing through a violation of the central regulation of hematopoiesis, possibly with the participation of the pituitary gland.

The pathogenesis of increased erythropoiesis cannot be explained oxygen starvation, as in reactive hypoxemic erythrocytosis; in particular, it was not possible to prove the worse return of oxygen, as if excessively fixed by hemoglobin in persons suffering from erythremia, it is also not possible to prove the breakdown of erythrocytes, which is lowered against the norm (blood breakdown is increased, but still lags behind colossal blood formation), or the constantly increased formation of hematopoietic substances by the stomach. substances (there are cases of a combination of erythremia with achilia of the stomach). Persistent functional impairment central regulation of hematopoiesis should be taken for most cases of erythremia. This explains the sometimes observed combination of erythremia with such diseases of neurogenic origin as hypertension or gastric ulcer. The pathogenesis of the disease determines the ways of therapeutic action on the central nervous system. As with leukemia, with erythremia, bone marrow tissue suffers with the development of excessive, not amenable to normal regulation of hematopoiesis; with erythremia, there is sometimes a leukemoid picture of white blood, and a sharp hyperthrombocytosis.

Raspberry-red bone marrow is anatomically found even in places of its complete inactivity in healthy individuals, spleen hyperplasia with accumulation huge amount erythrocytes. On the section, thrombosis is also usually found as the immediate cause of death.

Symptoms and signs of erythremia

At first glance, a characteristic cherry-red face with sharply injected, as if inflamed conjunctiva, cherry-colored lips, the same tongue, etc. (“erythrosis”) is striking. The cyanotic shade of the integument is due to a proportional increase in the reduced hemoglobin in capillary blood, despite the fact that hemoglobin saturation with oxygen in arterial blood may be normal. Patients are often overweight middle-aged or elderly people.

Complaints of patients are associated primarily with a violation cerebral circulation: headache, a feeling of heaviness, a feeling of fullness, fullness in the head, along with impaired vision, especially during mental work, which periodically becomes impossible; dizziness, a tendency to faint, tinnitus, memory impairment, irritability, general weakness, nosebleeds.

An objective examination of the organs often allows you to establish an increase in the spleen, usually moderate, rarely significant, sensitivity of the sternum (sternalgia). Blood circulation is disturbed a little, despite the increased mass of blood and viscosity, since there are no obstacles to blood flow in the periphery; arterial pressure fine; the blood flow is not slowed down, the heart is not dilated. X-ray examination detects increased blood supply to the lungs, and the study of the fundus reveals varicose veins, hemorrhages.
A blood test confirms the diagnosis of erythremia; the hemoglobin content is equal to 100% or more on the usual hemometer scale, the number of erythrocytes is 6,000,000 or more, with a moderately reduced color index (about 0.7).

The following changes in the blood are especially evident for erythremia:

• an increase in hematocrit to 60-70-80 or more instead of the normal 45;
• a significant increase in the mass of circulating blood - up to 8-10 liters instead of the normal 5 liters for an adult of average weight;
• a significant increase in blood viscosity - up to 8-10 units instead of the normal 4-5 compared to the viscosity of distilled water taken as a unit;
• signs of moderately elevated hemolysis and regeneration - increased blood levels of bilirubin, reticulocytes.

Irritation of the bone marrow can often be noted by the number of leukocytes, the number of which can reach 20,000-40,000 with a sharp neutrophilic shift (leukemoid reaction); the number of plates can also reach 500,000-750,000; such hyperthrombocytosis may contribute to thrombosis.

Urine is unremarkable, although albuminuria is not uncommon due to a general metabolic disorder or usually non-progressive dystrophic changes kidney tissue.

Basal metabolism is often increased by 15-20% against the norm.

Course, complications and clinical forms of erythremia

The onset of the disease is gradual, the course is progressive. For the most part, erythremia leads within 4-5 years to death from thrombosis of the cerebral, intra-abdominal vessels, from bleeding as a result of thrombosis, especially in the system portal vein, with the development of anemia and often also from cachexia, as in cancerous tumors. In less severe cases, patients live for decades, almost without experiencing painful symptoms. In the early stages of the disease, the spleen may not be enlarged; splenomegaly usually indicates an advanced form of the disease. vein thrombosis abdominal cavity accompanied by pain, fever, ascites, enlargement of the spleen with perisplenitis, hematemesis, melena. Under the name of erythroleukemia, there are forms of erythremia with a pronounced leukemoid reaction.

Clinically, it is extremely important to differentiate, along with a clinically independent form of the disease, symptomatic forms in tuberculosis of the spleen, with lesions diencephalon. Allocate another form of erythremia with cirrhosis of the liver; Availability hypertension with erythremia, it should be considered rather a coincidence, if there is not, as is more often the case, only some erythrocytosis in hypertensive patients without blood shifts typical for erythremia (thus, the allocation of a special hypertensive erythremia is little justified).

Diagnosis erythremia can be difficult at the beginning and in mild forms of the disease, when it is necessary to be guided by the already indicated blood shifts, as well as after major bleeding (usually from the portal vein system), when thrombophlebitis splenomegaly can be misdiagnosed in the presence of anemia.

Treatment of erythremia

Treatment of erythremia is desirable to be directed to the main disturbed mechanisms of regulation of hematopoiesis or the elimination of the main causes of the disease in various patients; however, in this respect, the essence of the disease is not well understood and appropriate treatment is of little value. There have been attempts to apply X-ray therapy of the brain, but without lasting success. In cases of association of erythremia with a specific infection - tuberculosis or syphilis - it is advisable to carry out specific treatment.

Basically, the treatment of erythremia is currently reduced to symptomatic measures aimed at increased breakdown or removal of blood and suppression of hematopoiesis. simple and effective method treatment of erythremia - systematic repeated bloodletting (500-700 ml 1-2 times a week; blood can be used if erythremia is associated with tuberculosis and other infections, for transfusion in patients with aplastic anemia). In the first days after bloodletting, blood clotting increases, hence there is some danger of thrombosis, to counteract which it is advisable to use leeches or hirudin and other anticoagulants. Small bloodletting can only stimulate hematopoiesis.

Erythremia is also treated by causing increased blood breakdown with hemolytic poisons, especially acetylphenylhydrazine, which is not indifferent, however, for parenchymal organs; large doses of arsenic in the form of Fowler's solution, apparently, can also reduce the number of red blood cells in erythremia. X-ray therapy in large doses, destroying young cells, is also a valid, although not indifferent, method of treating erythremia; in last years radioactive phosphorus is used for the same purpose. They try to limit blood formation and a diet, if possible, devoid of iron and meat; in some cases, if present at the same time peptic ulcer The stomach was resected in order to turn off the gastric hematopoietic factor. Oxygen therapy, apparently, is not justified, since there is no apoxemia in erythremia.

Splenectomy is contraindicated, since erythremia is a systemic disease and primarily of the bone marrow, while the spleen rather inhibits bone marrow hematopoiesis. However, in splenic tuberculosis with secondary erythremia, removal of the spleen may appear to be curative.

Erythremia is a persistent progressive increase in the concentration of red blood cells(erythrocytes) in the bloodstream due to a failure in the regulation of blood formation. AT medical literature given state also called Wakez-Osler disease, polycythemia vera. This blood disease refers to tumor diseases.

It is worth noting that in 2/3 of the cases, simultaneously with the increase in the level, also increase. Clinicians consider erythremia benign disease, but its prognosis is conditionally favorable. A patient with such a diagnosis can live long enough (taking into account full treatment). Death usually occurs due to the progression of various complications.

This blood disease is usually diagnosed in old age, but in recent times it is sometimes detected in people of working age. Representatives of the stronger sex suffer from it more often than women. Erythremia is a rare disease. In general, every year doctors diagnose it in 5 people out of 100 million.

Etiology

The exact causes of the progression of erythremia by scientists have not yet been established. But there are several predisposing factors that significantly increase the risk of progression of this disease:

• genetic predisposition. The reasons contributing to the progression of erythremia can be various mutations at the gene level. This fact is proven, since this blood disease is more often diagnosed in individuals suffering from certain genetic ailments. These include:, Klinefelter's syndrome;
• ionizing radiation. It can also be the cause of the development of erythremia, because the rays, penetrating the human body, cause damage at the gene level. As a result, certain cells may die or DNA will begin to mutate;
• toxic substances. Such substances, penetrating into the human body, can cause mutations at the genetic level. They are called mutagens. These include: azathioprine, benzene, chloramphenicol, cyclophosphamide.

Kinds

According to ICD-10 this disease blood belongs to the group. Clinicians distinguish two forms of the disease:

• acute, also called erythroleukemia;
• chronic.

Depending on the progression of blood pathology:

• true. There is a persistent increase in the concentration of red blood cells. This form is extremely rare in young children;
• relative or false. The peculiarity of this form is that the level of erythrocyte mass is normal, but the plasma volume gradually decreases (due to the loss of fluid by the body).

Depending on the pathogenesis, true polycythemia is divided into:

• primary;
• secondary.

stages

It is worth noting that at first erythremia absolutely does not manifest itself in any way. The general condition of the body does not suffer, as does the blood system. In view of the lack clinical signs, a person does not even suspect that such a dangerous disease is progressing in him.

Clinicians in the progression of erythremia note three stages:

• initial;
• erythremic;
• anemic.

Initial

It can last from a couple of months to several decades, and no symptoms will appear. There is a moderate increase in the concentration of red blood cells in the blood - from 5 to 7x10¹² per 1 liter of blood. It also increases the level.

erythremic

The level of red blood cells is constantly rising. Due to certain mutations, the tumor cell begins to differentiate into leukocytes and platelets. As a result of this pathological process blood volume increases - vessels and organs overflow. The blood itself also changes. It becomes viscous and the speed of its passage through the vessels is significantly reduced. Platelets are activated - they bind to each other and form specific plugs that block the lumen of small-caliber vessels. As a result, some parts of the body are no longer adequately supplied with oxygen and nutrients that the blood carries.

It is also worth noting that, as the concentration of cells in the blood increases, they are intensively destroyed in the spleen. It is also dangerous, because the products of their decay begin to actively enter the bloodstream.

anemic

It is characterized by the development of the process of fibrosis in the bone marrow - cells that are actively involved in the production of blood cells are gradually replaced fibrous tissue. As a result, the hematopoietic function gradually fades away and the level of leukocytes, platelets and erythrocytes in the bloodstream decreases. Extramedullary foci of hematopoiesis are formed in the spleen and liver. This is a kind of compensatory reaction, the main objective which is to normalize the concentration of blood cells.

This stage is the most dangerous, since its main symptom is prolonged bleeding(up to several hours). They pose a serious threat to human life.

Symptoms

Symptoms of erythremia directly depend on the stage of the disease. But it is also worth noting that some signs may appear on early stage and disappear until anemic.

Symptoms initial stage:

• hyperemia of the skin and mucous membranes. It is observed due to an increase in the concentration of red blood cells in the bloodstream. Hyperemia affects absolutely all parts of the body. Hyperemia can be mild, so the skin will not be red, but slightly pink. Many people take this shade as the norm and do not associate it with the progression of any disease;
• pain in the fingers. This symptom is manifested as a result of a violation of the normal circulation of blood in small-caliber vessels;
• headache. This symptom is not specific, but often manifests itself in the initial stage of erythremia.

Symptoms of the erythremic stage:

• increased hyperemia of the skin and mucous membranes;
• increase in blood pressure;
• erythromelalgia;
• pain in the joints;
• pruritus;
• finger necrosis;
• ulcers form on the gastrointestinal tract;
• thrombotic strokes;
• dilated;
• symptoms of iron deficiency: indigestion, delamination of the nail plates, dry skin, loss of appetite, the appearance of painful cracks in the corners of the mouth, and so on.

The third stage begins to progress due to the fact that the person has not been necessary treatment in the first and second stages. If the terminal stage has developed, then the prognosis is unfavorable - it can be fatal. The patient has the following symptoms:

• expressed. A person's skin turns pale, general weakness is noted, and there is a feeling of lack of air. He may faint;
• bleeding. They can appear spontaneously or even with minor trauma to the skin, articular joints or muscle structures. They represent a particular danger to the life of the patient, as they can last several hours.

Diagnostics

Diagnosis and treatment of this blood disease is carried out by a narrow specialist - a hematologist. For an accurate diagnosis, he resorts to laboratory and instrumental techniques diagnostics.

Medical therapy:

• myelobromol;
• hydroxyurea;
• aspirin;
• chimes;
• heparin;
• maltofer;
• ferrum Lek;
• allopurinol;
• anturan.

To reduce hemoglobin, they resort to erythrocytapheresis and bloodletting.

Symptomatic therapy:

• antihistamine drugs;
• antihypertensive agents;
• cardiac glycosides;
• gastroprotectors.

In order to correct anemia, doctors resort to blood transfusions. In order to correct the destruction of blood cells in the spleen, the organ is extracted (in case of ineffectiveness of conservative treatment methods).

If necessary, the patient is referred for a consultation with narrow specialists, such as an oncologist, rheumatologist, neurologist, gastroenterologist.

Treatment of pathology is carried out only in stationary conditions so that doctors can constantly monitor general state patient. If necessary, the treatment plan is adjusted. Since the disease is benign, with a full and timely therapy, a person can live a very for a long time. But if the treatment is absent, then the course of erythremia ends in death.

Erythremia is a disease that affects the hematopoietic system and is accompanied by an excess of the concentration of red blood cells in the blood. Erythremia is assigned to the ICD-10 code C94.

What is erythremia?

Erythremia - neoplastic disease blood, manifested mainly after the patient crosses the 40-50-year age limit. The disease is characterized by a long course and the appearance of complications on late stages. The aggravation of symptoms is associated with the transition of erythremia into a malignant form.

Erythremia classification

Depending on the nature of the course of the disease, acute or chronic erythremia is diagnosed.

Based clinical manifestations and the results of blood tests, the following stages of erythremia are distinguished:

• Initial. It develops over several (up to five) years. Asymptomatic or with mild symptomatic picture, manifested by a slight increase in the content of erythrocytes in the blood (no more than 7×10 12 /l).
• Erythremic (polycythemic). Progresses within 5-15 years. The concentration of erythrocytes in the blood reaches 8 × 10 12 / l (it is possible to exceed this indicator), the content of free hemoglobin increases, uric acid. Leukocytosis, thrombocytosis are detected, the risk of blockage of blood vessels by blood clots increases. Spleen, liver increase in size. As the erythremic stage develops, a decrease in the level of platelets in the blood is possible, accompanied by more frequent bleeding. Bone marrow scarring occurs.
• Anemic (terminal). It is found 10-20 years after the onset of the development of erythremia. The volume of cells produced by the bone marrow decreases to dangerous level due to replacement of bone marrow cells connective tissue. The functions of hematopoiesis are "shifted" to the spleen and liver, which are experiencing overload. Chronic erythremia accompanied by numerous disturbances in the vital activity of organs and systems.

Analyzing the factors influencing the symptoms of erythremia and the dynamics of changes in the erythrocyte mass, diagnose:

• true polycythemia. The concentration of red blood cells in the blood is proportional to the severity of the disease.
• False (relative) erythremia. Adverse symptomatic phenomena are not associated with an erythrocyte surge, but with a decrease in the volume of blood plasma due to dehydration, the harmful effects of other factors.

Reasons for the development of the disease

The exact causes of erythremia have not been determined. Doctors studying each of clinical cases take into account potential risk factors.

An increased likelihood of developing erythremia is due to:

• Genetic anomalies (gene mutations).
• The harmful effects of ionizing radiation ( higher doses receive patients working in the field nuclear energy undergoing courses of radiation anticancer therapy, living in the zone of abnormal radiation background).
• Ingestion of poisonous (toxic) substances.
• The systematic use of potent cytostatics.
• Severe diseases of the heart, lungs.

Despite the fact that the listed causes of erythremia are hypothetical, numerous medical research were able to explain why the disease provokes a number of complications. With erythremia, the regulatory mechanism for the production of blood cells is damaged (distorted) (their number becomes excessive). The blood flow is disturbed, tissue blood supply is destabilized, functional failures of varying severity occur.

Main signs and symptoms

The predominant symptomatology is determined by the stage of erythremia.

Signs of the initial stage

On the early stages pathology is asymptomatic. As erythremia develops, it manifests itself:

• A change in the shade (redness, pinking) of the mucous membranes, skin due to the accumulation of red blood cells in the vessels. The signs are mild, there are no complaints, so the diagnosis and treatment of erythremia is not carried out in every clinical case.
• . The appearance of the symptom is explained by the destabilization of cerebral circulation.
• Pain in fingers. Unpleasant symptoms arise as a result of a violation of the process of transporting oxygen to tissues, ischemia.

Manifestations of the erythremic stage

The erythremic stage is accompanied by:

• Painful sensations in the joints (explained by the accumulation of decay products of blood cells in the joint tissues).
• Enlargement of the spleen, liver.
• Hypertensive symptoms.
• Redness, itching skin, intensified by contact with water.
• Expansion of blood vessels.
• Necrosis of fingers.
• Increased bleeding (after dental interventions, violations of the integrity of the skin).
• Exacerbations of organ ulcers digestive tract(associated with a decrease in the barrier functions of the mucous membranes against the background of circulatory disorders). Patients complain of nausea, vomiting, heartburn, pain in the abdomen.
• An increase in the liver (a consequence of overflow of the organ with blood), pain, heaviness in the right hypochondrium, digestive failures, respiratory disorders.
• Anemic symptoms (distortion of taste perception, digestive disorders, exfoliation of the nail plates, brittle hair, overdrying of the mucous membranes and skin, the appearance of cracks in the corners of the mouth, a decrease in resistance to infectious agents).
• that interfere with the transport of blood.
• Malfunctions in the functioning of the myocardium, stretching of the heart, arrhythmia, stroke, heart attack (formed blood clots clog the vascular lumens, brain and heart tissues experience acute deficiency nutrients and oxygen).

End stage symptoms

A common manifestation of the most severe stage of erythremia is bleeding that occurs without exposure to external factors or as a result of injury to muscle, joint tissues, skin. Key reason development of bleeding in erythremia is a significant decrease in the concentration of platelets in the blood.

Against the background of progressive anemia, the state of health worsens, which is manifested by sensations of lack of air (even with minimum loads), fatigue, weakness, fainting, blanching of the mucous membranes and skin.

In the absence of effective therapeutic measures available fatal outcome.

What is included in the diagnosis of erythremia

Scroll diagnostic procedures prescribed by a hematologist is determined by the clinical picture of erythremia, the results of the examination and the study of the anamnesis.

A blood test is the primary examination performed when a diagnosis of erythremia is suspected. To get the most reliable results sampling of biomaterial is carried out on an empty stomach in morning time days.

Leukocytes, erythrocytes, hemoglobin, hematocrit increase by middle stage, decreasing at the anemic stage. ESR decreases at the erythremic stage, characterized by increased rates at the terminal stage of erythremia.

When conducting blood biochemistry, a decrease in the concentration of iron, an increase in the content of uric acid are detected (the more severe the stage of erythremia, the more significant the deviation from the norm). Perhaps a slight increase in bilirubin blood counts, aspartate aminotransferase, alanine aminotransferase.

Assessment of the iron-binding capacity of the blood serum helps to determine the progressive stage of the disease. At the middle stage of erythremia, a significant increase in the TIBC parameter is found. If the anemic stage of erythremia is accompanied by bleeding, the rate decreases. Bone marrow fibrosis is characterized by an increase in TI.

An enzyme-linked immunosorbent assay is performed to calculate the concentration of erythropoietin (a hormone that activates the production of red blood cells) in the blood. Apply a solution containing antibodies to the antigen of the hormone (erythropoietin). After counting the amount of antigen, the content of erythropoietin is estimated.

Puncture of the bone marrow

Puncture for erythremia is carried out to obtain maximum information about the state of the bone marrow. In the bone (rib, pelvic bone, vertebral process or sternum) a hollow needle is inserted, the material is taken, studying it with a microscope.

The procedure is accompanied painful sensations, the risk of damage internal organs located in the puncture zone. To avoid distortion of the results of the puncture, the procedure is carried out without the use of painkillers. To prevent infection, disinfection (sterilization) of the puncture site is carried out before and after the sampling of bone biomaterial.

During microscopic analysis, the number of each of the subtypes of blood cells is counted, cancer cells are detected, and foci of pathological growth of connective tissue are identified.

An indication for the examination is an increase in the size of the liver or spleen. Erythremia is accompanied by:

• Accumulation of blood in organs.
• Identification of foci of hyperechogenicity (a sign of fibrotic processes).
• Heart attacks (affected areas correspond to cone-shaped areas of increased echogenicity).

dopplerography

With the help of ultrasonic waves, the speed of blood flow, the severity of a heart attack, stroke are determined. Areas where blood circulation is disturbed or absent are found.

If necessary, the complex of examinations is supplemented by the study of the fundus, radioisotope, differential diagnosis erythremia (to exclude arterial hypoxemia, kidney disease, various forms myelosis).

Modern treatment and traditional medicine

The prognosis and the likelihood of developing complications of the disease depend on how timely the treatment of erythremia is started.

Drug therapy

In the treatment of erythremia, apply:

• Antitumor complexes (cytostatics). Contribute to the destruction of mutated blood cells. The prerequisites for undergoing a treatment course are thrombosis, heart attack, stroke, enlargement of the liver, spleen, critical (significantly exceeding the permissible values) indicators of the content of blood cells. The duration of the course of treatment, accompanied by constant control the patient's condition ranges from several weeks to several months.
• Vasodilators, anti-inflammatory drugs, drugs that inhibit blood clotting factors. Provides increased blood flow.
• (complete the deficiency of the microelement).
• Medicines that disrupt the production of uric acid, accelerating its excretion from the body with urine. The result is the elimination of exacerbations of articular symptoms.
• Drugs that normalize blood pressure (with hypertension).
• Antihistamine complexes. Contribute to the elimination of itching and other allergic manifestations.
• Cardiac glycosides that normalize the vital activity of the heart.
• Gastroprotectors that minimize the likelihood of developing stomach ulcers.

Blood flow correction

Improve clinical picture erythremia is helped by phlebotomy (bleeding), during which up to 300-400 ml is removed from the bloodstream. blood. The procedure carried out according to the method of sampling venous blood, helps to optimize blood circulation, reduce blood viscosity, normalize blood composition, pressure indicators, improve the condition of blood vessels.

Erythrocytapheresis, used as an alternative to phlebotomy, involves passing blood through a machine that removes red blood cells from it, and then returning the blood to the body. The regularity of the manipulation - weekly (course - 3-5 procedures).

If necessary, donated blood is transfused.

In the absence of the effect of conservative methods of therapy on severe stages erythremia, an operation is performed to remove the spleen affected by erythremia.

Traditional medicine

To improve the condition of patients suffering from erythremia, with the permission of the doctor, it is allowed to use:

• flower juice horse chestnut(reduces the intensity of thrombosis).
• Tincture from medicinal sweet clover(normalizes blood pressure, eliminates sleep disorders, manifestations of migraine).
• Decoctions based on the burial ground, hornbeam grass, nettle, periwinkle (improve the condition of blood vessels, optimize blood circulation).
• Alcohol tincture on goat willow bark (helps reduce hemoglobin in the blood).

The regimens for taking each of the herbal remedies must be agreed with a medical specialist.

To obtain an effect similar to phlebotomy (erythrocytapheresis), hirudotherapy is carried out, which helps to reduce the volume and optimize the composition of the circulating blood.

Pediatric erythremia

Erythremia in children is much less common than in adult (old) age. The list of potential risk factors for the development of erythremia includes dehydration (a consequence of burns, intoxication), hypoxia, heart defects, and pulmonary ailments.

Parents should be closely monitored for symptoms of fatigue, general weakness, sleep disturbances, redness, blueness, bleeding of the skin and mucous membranes, skin itching, changes in the shape of the fingers, taking on the appearance drumsticks. At the first manifestations of erythremia, you should consult a pediatrician.

In addition to strictly following treatment course, it is important not to allow physical, psycho-emotional overload, to spend maximum time on fresh air except sunbathing.

Possible Complications

Changes in the composition of the blood that accompany erythremia provoke complications:

• Peptic ulcer.
• Stroke.
• Infarction of the spleen, heart.
• Cirrhosis of the liver.
• Depletion of the body.
• Urolithiasis, gallstone disease.
• Gout.
• Nephrosclerosis.
• Severe forms of anemia that require constant blood transfusions.
• Malignant degeneration of erythremia.

Nutrition Features

Diet food is one of important elements medical procedures. The diet for erythremia is aimed at thinning the blood, minimizing the production of red blood cells, which has a positive effect on the patient's condition.

• Alcohol that destroys tissues and organs.
• Fruit and vegetable dishes (based on fruits of red shades).
• Food rich in preservatives, dyes.
• Beef, liver.
• Fish dishes.
• products rich in ascorbic acid(with high hemoglobin blood counts).
• Simple (refined) carbohydrates.

The diet is allowed to include:

• Whole grain products.
• Eggs.
• Dairy products (with low fat content).
• White beans.
• Greens.
• Nuts.
• Green tea.
• Raisins, dried apricots (in limited quantities).

Prevention and prognosis

Absence specific prevention erythremia is explained by incomplete knowledge of the mechanisms of the development of the disease. Recommendations of specialized specialists are reduced to:

• I refuse bad habits, consumption of medicines without the consent of medical specialists.
• Limit exposure to the sun.
• Prevention of exposure to harmful types of radiation.
• Strengthening the immune system.
• Maintaining water balance.
• Increasing motor activity.
• Carrying out diagnostics (including self-diagnosis of erythremia) aimed at timely detection pathology.

The prognosis for life expectancy depends on the stage at which erythremia is identified, as well as on the timeliness and effectiveness of treatment procedures. With a clear implementation of the doctor's instructions regarding the treatment of erythremia, diet, lifestyle, the prognosis is favorable: life expectancy is 20 years or more from the moment the disease was diagnosed.

erythremia

erythremia (or true polycythemia) is a tumor of the blood system that arises from the precursor cell of myelopoiesis. Erythremia is a variant of chronic leukemia and is considered the most benign blood tumor. In other words, erythremia proceeds for a long time and leads to complications rather late.

Causes

The cause of the development of erythremia remains unknown. Currently, the virus-genetic theory of the development of the disease is considered the most common. According to this theory, some types of viruses are able to enter the body and infect immature cells in the bone marrow or lymph nodes. In the future, infected cells begin to divide frequently, which leads to the onset of the tumor process.

Concerning hereditary causes erythremia, then it is questionable, since this disease often runs in families.

Predisposing factors for the development of erythremia can be: radiation, substances contained in varnishes and paints, certain types of viruses, intestinal infections, tuberculosis, surgical interventions, stress.

Symptoms

The main symptoms of erythremia are:

plethoric syndrome: headaches and dizziness, pain behind the sternum with physical activity, erythrocyanosis, "rabbit eyes" (redness of the eyes), pruritus, increased blood pressure, erythromelalgia, heaviness in the left side of the abdomen due to an enlarged spleen;

myeloproliferative syndrome: bone pain, heaviness in the left and upper abdomen;

fatigue and general weakness;

increase in body temperature.

Diagnostics

Diagnosis of erythremia includes:

analysis of the anamnesis and complaints of the patient;

physical examination;

·blood analysis;

·blood chemistry;

·Analysis of urine;

research of the bone marrow;

trephine biopsy;

Ultrasound examination of internal organs;

spiral computed tomography;

·Magnetic resonance imaging.

Types of disease

Erythremia proceeds in several stages:

I. Initial stage lasting 5 years or more.

II. Polycythemic stage with further myeloid metaplasia of the spleen. Lasts from 5 to 15 years.

III. Anemia stage - develops 15-20 years after the onset of the disease.

Patient's actions

Appearance anxiety symptoms requires immediate medical attention. When confirming the diagnosis of "erythremia", the patient should strictly adhere to the doctor's recommendations and undergo the necessary courses of treatment.

Treatment

In order to normalize the number of blood cells, bloodletting is practiced. As a rule, 200 to 400 ml of blood is extracted. Before the bloodletting procedure, the patient is prescribed special drugs that improve blood flow and reduce its clotting.

Removal of excess red blood cells is possible with the help of erythrocytepheresis - a way to purify the blood outside the body.

Chemotherapy is carried out at the second stage, in the presence of myeloid metaplasia of the spleen. With a pronounced increase in the spleen (due to the occurrence of a tumor process in it), radiation therapy is indicated.

Symptomatic therapy of erythremia involves the use antihypertensive drugs, antihistamines, antiplatelet agents, anticoagulants, hemostatic and diuretic drugs.

At the third stage of the disease, transfusion of blood components is indicated. A transfusion of erythrocyte and platelet (with a significant decrease in the number of platelets) mass is performed.

Complications

Complications with erythremia can be:

Thrombosis, which can occur in various parts of the body;

Bleeding that occurs with any (even small) surgical interventions. AT rare cases possible occurrence of spontaneous bleeding, in particular from the vessels gastrointestinal tract and from hemorrhoids;

urolithiasis and gout;

Lack of treatment can lead to the death of the patient 18 months after diagnosis. Treatment can increase life expectancy by 7-15 years. Most often, the patient dies due to thrombosis, in rare cases - due to bleeding and the development of secondary tumors.

Prevention

There is no way to prevent erythremia yet.

Erythremia is a tumor process hematopoietic system which is a type of chronic leukemia. The disease is characterized by an increase in the number of red blood cells, as well as an increase in the number of platelets and leukocytes. This condition causes an increase in hemoglobin in the blood, which leads to an increase in the volume of circulating blood.

Treatment of erythremia is important to start at an early stage of the disease, when its symptoms have just been identified and confirmed. At modern diagnostics there is no problem with that. Then later a person will not have to worry about life forecasts and panic because erythremia has a lot in common with oncological diseases.

Symptoms of blood erythremia

The disease has three stages: initial, erythremic and anemic. Each of these stages is characterized by a peculiar symptomatology.

initial stage . In this phase, the following symptoms may appear: redness of the skin and mucous membranes, headaches, discomfort of the fingers and toes. The spleen is slightly enlarged. The duration of the stage is five years.

erythremic stage . On palpation of the spleen, it is markedly enlarged. The blood becomes more viscous, its clotting is disturbed. Symptoms of the disease become aggressive and are expressed in the following states:

• weakness, headaches;
• itching of the skin after contact with water;
• necrosis of the tissues of the upper and lower extremities;
• pain in the fingertips;
• myocardial infarction;
• stroke;
• deep vessel thrombosis;
• cardiac ischemia.

The average duration of the disease is 10 years, after which erythremia becomes more severe.

anemic stage . Increased risk of stroke, myocardial infarction and acute thrombosis. This stage is dangerous because it has complications:

• acute leukemia;
• myeloid leukemia;
• hypoplastic changes in the hematopoietic system.

The blood formula in patients is shifted: the analysis indicates erythrocythemia, neutrophilia and panmyelosis.

Erythremia and cancer: is there a link?

Erythremia or chronic polycythemia chronic leukemia, which can be benign or malignant in nature and affect the blood system. Many associate the word "leukemia" with the phrase "blood cancer". In fact, erythremia affects the hematopoietic tissue, while cancerous tumors affect only connective tissues.

Polycythemia has a chronic benign course that lasts for years. However, at any moment a gross change in erythropoiesis may occur, as a result of which the disease takes a sharp progression and passes into malignant stage. It is very important to detect the disease in time, because when active treatment cancer may not develop.

Treatment: from bloodletting to chemotherapy

A disease such as erythremia requires treatment in a hospital where there is a hematological profile. The tactics of therapy depends on many factors: the general condition of the patient, the stage of development of the disease and hematological parameters. Treatment of erythremia is usually carried out by the following methods:

• bloodletting. This method is the oldest and, despite this, it is widely used in the treatment of polycythemia. The essence is simple: with the help of bloodletting, the amount of red blood cells and hemoglobin (up to 150 g / l) in human blood is reduced.
• treatment aimed at normalizing indicators in the blood formula (hydroxycarbamide, hydroxyurea, myelosan);
• antiplatelet therapy (aspirin, dipyridamole);
• Interferon-α2b with cytotoxic drugs (chemotherapy).

Each patient is assigned individual treatment, because many drugs have a lot of contraindications, which can lead to complication and aggravation of the disease.

Folk remedies for erythremia

On the this moment ethnoscience haven't found funds yet. that are able to treat erythremia. Many doctors are sure that if treated only non-traditional methods, then this will not bring an effect, and the patient will only lose precious time. Erythremia can be treated in a complex way, that is, at the same time, medical and traditional medicine can be used.

Popular folk remedy- this is alcohol tincture goat willow tree bark, which can reduce hemoglobin, thin the blood and cleanse the blood vessels. It is prepared in the following way. The bark is poured with alcohol 1: 1 and placed in a dark, dry place. After 2-3 days, it can be taken as a medicine, one tablespoon three times a day. This treatment is not suitable for everyone, so before using the tincture, you need to consult with your doctor.

Forecast for life

Despite the fact that erythremia is considered a “good” cancer and has a benign course, with untimely treatment the disease is usually fatal. Patients with erythremia can live up to 20 years. What criteria determine the prognosis of the disease:

• timely and proper treatment increases life expectancy;
• treating symptoms improves quality of life;
• the level of erythrocytes, platelets and leukocytes in the blood formula: the higher the indicators, the worse the outcome for the patient;
• body's response to therapy. It happens that erythremia is poorly treatable or does not respond at all;
• severity of bone marrow damage;
• thrombolytic complications. The disease very often causes myocardial infarction, stroke and thrombosis of internal organs, so death can occur from complications caused by erythremia;
• with absence acute leukemia life expectancy is increasing.

It is very important not to waste time at the first signs of illness and in no case be afraid to hear terrible diagnosis. If during the period of illness strictly adhere to medical advice, you can live for a long time.