Chronic erythremia. Early signs and mature symptoms of the disease. Treatment of erythremia at home

A relatively long and benign course is characteristic, which does not exclude, however, malignant transformation into acute leukemia with a fatal outcome.


Erythremia is considered a fairly rare disease and occurs with a frequency of 4-7 cases per 100 million population per year. It affects mainly middle-aged and elderly people ( over 50 years old), however, cases of illness have been reported in more than early age. Men and women get sick with the same frequency.

Interesting Facts

• RBCs make up about 25% of all cells human body.
• Every second in bone marrow about 2.5 million new red blood cells are formed. Approximately the same amount is destroyed throughout the body.
• The red color of red blood cells is given by iron, which is part of hemoglobin.
• Erythremia is one of the most benign tumor processes in the blood.
• Erythremia can be asymptomatic for many years.
• Patients with erythremia are prone to profuse bleeding, despite the fact that the platelet count ( responsible for stopping bleeding) increased.

What are erythrocytes?

Structure and function of erythrocytes

The normal number of red blood cells in the blood varies by gender.

The norm of erythrocytes is:

• in women - 3.5 - 4.7 x 10 12 in 1 liter of blood;
• in men - 4.0 - 5.0 x 10 12 in 1 liter of blood.

The process of transporting gases occurs as follows:

• in the pulmonary capillaries finest blood vessels) an oxygen molecule joins the iron, which is part of hemoglobin ( an oxidized form of hemoglobin is formed - oxyhemoglobin).
• From the lungs with blood flow, erythrocytes are transferred to the capillaries of all organs, where the oxygen molecule is separated from oxyhemoglobin and transferred to the cells of body tissues.
• Instead, carbon dioxide released by tissues joins hemoglobin ( a complex called carbhemoglobin is formed).
• When erythrocytes containing carbhemoglobin pass through the pulmonary capillaries, carbon dioxide is detached from hemoglobin and released with exhaled air, and in return another oxygen molecule is added and the cycle repeats.

The norm of hemoglobin is:

• in women - 120 - 150 grams / liter;
• in men - 130 - 170 grams / liter.

Where are erythrocytes formed?

Starting from the 4th month of embryonic development, foci of hematopoiesis appear in the red bone marrow, which is the main hematopoietic organ after the birth of a child and throughout life. Its total volume in an adult is about 2.5 - 4 kg and is distributed in various bones of the body.

In an adult, the red bone marrow is located:

• in the pelvic bones 40% );
• in the vertebrae ( 28% );
• in the bones of the skull 13% );
• in the ribs ( 8% );
• in the long bones of the arms and legs ( 8% );
• in the chest ( 2% ).

How are erythrocytes formed?

When a stem cell divides, it produces:

• Progenitor cells of myelopoiesis. Depending on the needs of the body, they can divide with the formation of one of the blood cells - an erythrocyte, a platelet ( responsible for stopping bleeding) or leukocyte ( protecting the body from infections).
• Progenitor cells of lymphopoiesis. They form lymphocytes that provide immunity ( protective function).

This process includes several successive divisions, as a result of which the cell decreases in size, loses the nucleus and accumulates hemoglobin.

For the formation of red blood cells are necessary:

• Vitamins. Vitamins such as B2 ( riboflavin), AT 6 ( pyridoxine), AT 12 ( cobalamin) and folic acid are essential for the formation of normal blood cells. With a lack of these substances, the processes of division and maturation of cells in the bone marrow are disrupted, as a result of which functionally incompetent erythrocytes are released into the bloodstream.
• Iron. This trace element is part of hemoglobin and plays a major role in the process of transporting oxygen and carbon dioxide by red blood cells. The intake of iron in the body is limited by the rate of its absorption in the intestine ( 1 - 2 milligrams per day).

As a result of an increase in the number of red blood cells in the blood, oxygen delivery to the tissues improves. This inhibits the excretion of erythropoietin by the kidneys and reduces its effect on the myelopoiesis progenitor cell, which inhibits the formation of red blood cells in the red bone marrow.

How are red blood cells destroyed?

In the process of destruction of the erythrocyte, iron, which is part of hemoglobin, is released into the bloodstream and transported by special proteins to the red bone marrow, where it again takes part in the formation of new erythrocytes. This mechanism is extremely important for maintaining the process of hematopoiesis, which normally requires from 20 to 30 milligrams ( mg) iron per day ( while only 1-2 mg is absorbed with food).

What is erythremia?

The emergence and development of erythremia

The mutant precursor cell of myelopoiesis begins to multiply intensively, passing through all the stages of growth and development described above, and the result of this process is the appearance of absolutely normal and functionally viable erythrocytes in the blood.

Thus, two different types of erythrocyte precursor cells appear in the red bone marrow - normal and mutant. As a result of intensive and uncontrolled formation of erythrocytes from a mutant cell, their number in the blood increases significantly, exceeding the needs of the body. This, in turn, inhibits the release of erythropoietin by the kidneys, which leads to a decrease in its activating effect on the normal process of erythropoiesis, but does not affect the tumor cell in any way.

In addition, as the disease progresses, the number of mutant cells in the bone marrow increases, which leads to the displacement of normal hematopoietic cells. As a result, there comes a moment when all ( or almost all) erythrocytes of the body are formed from a tumor clone of the myelopoiesis precursor cell.

With erythremia, most of the mutant cells turn into erythrocytes, but a certain part of them develop in a different way ( with the formation of platelets or leukocytes). This explains the increase not only in erythrocytes, but also in other cells, the precursor of which is the parent cell of myelopoiesis. As the disease progresses, the number of platelets and leukocytes produced from the tumor cell also increases.

The course of erythremia

In the development of erythremia conditionally distinguish:

• initial stage;
• erythremic stage;
• anemic ( terminal) stage.

erythremic stage
It is characterized by an increase in the number of red blood cells formed from an abnormal progenitor cell ( more than 8 x 10 12 in 1 liter of blood). As a result of further mutations, the tumor cell begins to differentiate into platelets and leukocytes, causing an increase in their number in the blood.

The consequence of these processes is the overflow of blood vessels and all internal organs. The blood becomes more viscous, the speed of its passage through the vessels slows down, which contributes to the activation of platelets directly in the vascular bed. Activated platelets bind to each other, forming the so-called platelet plugs, which clog the lumen of small vessels, disrupting the flow of blood through them.

In addition, as a result of an increase in the number of cells in the blood, their destruction in the spleen is enhanced. The result of this process is an excessive intake of cellular decay products into the blood ( free hemoglobin, purines).

anemic stage
With the progression of the disease in the bone marrow, the process of fibrosis begins - the replacement of hematopoietic cells with fibrous tissue. The hematopoietic function of the bone marrow gradually decreases, which leads to a decrease in the number of erythrocytes, leukocytes and platelets in the blood ( up to critical values).

The result of this process is the appearance of extramedullary foci of hematopoiesis ( outside the bone marrow) in the spleen and liver. This compensatory reaction is aimed at maintaining a normal number of blood cells.

In addition, the appearance of extramedullary foci of hematopoiesis may be due to the release of tumor cells from the bone marrow and their migration with the blood flow to the liver and spleen, where they linger in the capillaries and begin to multiply intensively.

Causes of erythremia

The occurrence of erythremia can contribute to:

• genetic predisposition;
• ionizing radiation;
• toxic substances.

genetic predisposition

The risk of developing erythremia is increased with:

• Down Syndrome - a genetic disease manifested by a violation of the shape of the face, neck, head and a developmental delay in the child.
• Klinefelter syndrome - a genetic disease that manifests itself during puberty and is characterized by disproportionate development of the body ( tall, long and thin arms and legs, long waist), as well as possible mental disabilities.
• Bloom's Syndrome a genetic disease characterized by short stature, hyperpigmentation of the skin, disproportionate development of the face and a predisposition to tumors of various organs and tissues.
• Marfan syndrome - genetic disease that interferes with the development connective tissue body, which is manifested by high growth, long limbs and fingers, impaired vision and of cardio-vascular system.

ionizing radiation

People in the explosion zones are most exposed to radiation. atomic bombs, catastrophes at nuclear power plants, as well as patients with malignant tumors, in whom various methods of radiotherapy were used as treatment.

Toxic Substances

Chemical mutagens that cause erythremia are:

• Benzene - is part of gasoline, chemical solvents.
• Cytotoxic drugs - azathioprine, methotrexate, cyclophosphamide.
• Antibacterial drugs - chloramphenicol ( chloramphenicol).

Symptoms of erythremia

Symptoms of the initial stage

• Redness of the skin and mucous membranes. Occurs as a result of an increase in the number of red blood cells in the vessels. Redness is noted in all parts of the body, in the head and limbs, oral mucosa, and eye membranes. At the initial stage of the disease this symptom can be mildly expressed, as a result of which a pinkish tint of the skin is often taken as the norm.
• Pain in fingers and toes. This symptom is due to a violation of blood flow through small vessels. At the initial stage, this is largely due to increased blood viscosity due to an increase in the number of cellular elements. Violation of oxygen delivery to organs leads to the development of tissue ischemia ( oxygen starvation), which is manifested by bouts of burning pain.
• Headache. A non-specific symptom, which, however, can be very pronounced in the initial stage of the disease. Frequent headaches can occur as a result of impaired blood circulation in the small vessels of the brain.

Symptoms of the erythremic stage

Symptoms of the erythremic stage are:

• redness of the skin;
• necrosis of fingers and toes;
• increase in blood pressure;
• liver enlargement;
• enlargement of the spleen;
• skin itching;
• increased bleeding;
• symptoms of iron deficiency;
• thrombotic strokes;
• dilated cardiomyopathy.

Erythromelalgia
The reasons for this phenomenon have not been precisely established. It is believed that it is associated with impaired blood circulation in small peripheral vessels. It is manifested by sudden bouts of redness and acute, burning pain in the area of ​​​​the tips of the fingers and toes, earlobes, and the tip of the nose.

Usually the lesion is bilateral. Attacks can last from several minutes to several hours. Some relief comes when the affected area is immersed in cold water. As the underlying disease progresses, the soreness zones may increase, spreading to the hand and foot.

Necrosis of fingers and toes
An increase in the number of platelets formed ( characteristic of this stage of the disease), as well as a slowdown in blood flow due to an increase in its viscosity, contributes to the formation of platelet plugs that clog small arteries.

The described processes lead to local circulatory disorders, which is clinically manifested by pain, which is subsequently replaced by a violation of sensitivity, a decrease in temperature and tissue death in the affected area.

Increase in blood pressure
It occurs due to an increase in the total volume of circulating blood ( BCC) in the vascular bed, as well as as a result of increased blood viscosity, which causes an increase in vascular resistance to blood flow. Blood pressure rises gradually as the disease progresses. Clinically, this can be manifested by increased fatigue, headaches, visual impairment and other symptoms.

Liver enlargement ( hepatomegaly)
The liver is an extensible organ, normally depositing up to 450 milliliters of blood. With an increase in BCC, the blood overflows the hepatic vessels ( more than 1 liter of blood can be retained in it). With the migration of tumor cells to the liver or with the development of foci of extramedullary hematopoiesis in it, the organ can reach enormous sizes ( ten or more kilograms).

Clinical manifestations of hepatomegaly are heaviness and pain in the right hypochondrium ( arising from excessive stretching of the liver capsule), digestive disorders, respiratory failure.

Enlargement of the spleen ( splenomegaly)
Due to the increase in BCC, the spleen overflows with blood, which eventually leads to an increase in the size and compaction of the organ. Also, this process is facilitated by the development of pathological foci of hematopoiesis in the spleen. In an enlarged organ, the processes of destruction of blood cells occur more intensively ( erythrocytes, platelets, leukocytes).

Skin itching
The appearance of this symptom is caused by the influence of a special biological active substance- histamine. Under normal conditions, histamine is contained in leukocytes and is released only under certain pathological conditions, often of an allergic nature.

With a long course of the disease, there is an increase in the number of leukocytes formed from the tumor cell. This leads to their more intensive destruction in the spleen, as a result of which a large number of free histamine, which, among other effects, causes severe itching, aggravated by contact with water ( when washing hands, taking a bath, being exposed to rain).

Increased bleeding
It can occur both as a result of an increase in blood pressure and BCC, and as a result of excessive activation of platelets in the vascular bed, which leads to their depletion and disruption of the blood coagulation system. Erythremia is characterized by prolonged and profuse bleeding from the gums after tooth extraction, after small cuts and injuries.

Joint pain
Due to the increased destruction of blood cells, a large number of their decay products enter the bloodstream, including purines, which are part of nucleic acids ( the genetic apparatus of cells). Under normal conditions, purines are converted to urates ( uric acid salts) that are excreted in the urine.

With erythremia, the amount of urate formed is increased ( urate diathesis develops), as a result of which they settle in various organs and tissues. Over time, they accumulate in the joints ( first in small ones, and then in larger ones.). Clinically, this is manifested by redness, soreness, and limited mobility in the affected joints.

Ulcers of the gastrointestinal system
Their occurrence is associated with a violation of blood circulation in the mucous membrane of the stomach and intestines, which significantly reduces its barrier function. As a result, acidic gastric juice and food ( especially sharp or rough, poorly processed) injures the mucous membrane, contributing to the development of ulcers.

Clinically, this condition is manifested by abdominal pain that occurs after eating ( with stomach ulcer), or on an empty stomach ( with duodenal ulcer). Other manifestations are heartburn, nausea and vomiting after eating.

Symptoms of iron deficiency
As mentioned earlier, the amount of iron that enters the body with food is limited by the rate of its absorption in the intestine and is 1-2 mg per day. Under normal conditions, the human body contains 3-4 grams of iron, while 65-70% is part of hemoglobin.

With erythremia, most of the iron entering the body ( up to 90 – 95%) is used to form red blood cells, resulting in a lack of this element in other organs and tissues.

Iron deficiency symptoms are:

• dry skin and mucous membranes;
• thinning and increased fragility of hair;
• cracks in the corners of the mouth;
• delamination of nails;
• lack of appetite;
• indigestion;
• violation of taste and smell;
• decreased resistance to infections.

myocardial infarction
The mechanism of occurrence of a heart attack is the same as in a stroke - the resulting blood clots can clog the lumen blood vessels feeding the heart. Since the compensatory capabilities of this organ are extremely small, oxygen reserves are very quickly depleted in it, which leads to necrosis of the heart muscle.

A heart attack is manifested by a sudden onset of severe, acute pain in the region of the heart, which lasts more than 15 minutes and can spread to the left shoulder and back to the left. This condition requires urgent hospitalization and the provision of qualified medical care.

Dilated cardiomyopathy
This term refers to a violation of the heart, associated with an increase in the volume of circulating blood. When the chambers of the heart are overfilled with blood, the organ is gradually stretched, which is a compensatory reaction aimed at maintaining blood circulation. However, the compensatory capabilities of this mechanism are limited, and when they are depleted, the heart stretches too much, as a result of which it loses the ability to normal, full-fledged contractions.

Clinically, this condition is manifested by general weakness, increased fatigue, pain in the heart area and disorders heart rate, edema .

Symptoms of the anemic stage

The main manifestations terminal stage erythremia are:

• bleeding;

The occurrence of bleeding in the terminal stage is due to:

• a decrease in the formation of platelets;
• formation of functionally incompetent platelets.

The causes of anemia in the terminal stage of the disease can be:

• Inhibition of hematopoiesis in the bone marrow. The reason for this is the proliferation of connective tissue ( myelofibrosis), which completely displaces hematopoietic cells from the bone marrow. As a result, the so-called aplastic anemia develops, manifested by a decrease in the level of red blood cells, platelets and white blood cells.
• iron deficiency. The lack of this microelement leads to a violation of the formation of hemoglobin, as a result of which large, functionally incompetent erythrocytes enter the bloodstream.
• Frequent bleeding. In this case, the rate of formation of new blood cells is insufficient to make up for the losses that occur during bleeding. This situation is further exacerbated by iron deficiency.
• Increased destruction of red blood cells. In the enlarged spleen, a large number of red blood cells and platelets are retained, which are destroyed over time, causing the development of anemia.

• pale skin and mucous membranes;
• general weakness;
• increased fatigue;
• feeling of lack of air ();
• frequent fainting.

Diagnosis of erythremia

• bone marrow puncture;
• laboratory markers;
• instrumental research.

General blood analysis

Blood sampling for analysis is carried out in the morning, on an empty stomach, in a specially equipped room. As a rule, blood is taken from the ring finger of the left hand. Having previously treated the fingertip with cotton wool moistened with alcohol, the skin is punctured to a depth of 2–4 mm with a special needle. The first drop of blood formed is wiped off with a cotton swab, after which several milliliters of blood are drawn into a special pipette.

The resulting blood is transferred into a test tube and sent to the laboratory for further analysis.

The main parameters studied in the KLA are:

• The number of blood cells. The number of erythrocytes, platelets and leukocytes is counted separately. Based on the number of cells in the test material, conclusions are drawn about their number in the vascular bed.
• The number of reticulocytes. Their number is determined in relation to the total number of erythrocytes and is expressed as a percentage. Gives information about the state of hematopoiesis in the bone marrow.
• The total amount of hemoglobin.
• Color index. This criterion allows you to determine the relative content of hemoglobin in the erythrocyte. Normally, one erythrocyte contains from 27 to 33.3 picograms ( pg) hemoglobin, which is characterized by a color index of 0.85 - 1.05, respectively.
• Hematocrit. Displays the proportion of cellular elements in relation to the total blood volume. Expressed as a percentage.
• Sedimentation rate of erythrocytes ( ESR). The time during which the separation of blood cells and plasma will occur is determined. The more red blood cells in the blood volume, the stronger they repel each other ( due to negatively charged surfaces cell membranes ), and the slower the ESR will be.

Changes in the general blood test, depending on the stage of erythremia

Blood chemistry

Blood sampling for biochemical analysis is performed from the ulnar or radial saphenous veins of the arm, located on the anterior surface of the elbow bend. The patient sits on a chair and puts his hand on his back. The nurse bandages the patient's arm with a tourniquet 10-15 cm above the elbow bend, and asks him to "work with his fist" - clench and unclench his fingers ( this will increase blood flow to the veins and make the procedure easier).

Having determined the location of the vein, the nurse carefully treats the site of the future puncture with cotton wool soaked in alcohol, and then inserts a needle connected to the syringe into the vein. After making sure that the needle is in the vein, the nurse removes the tourniquet and draws a few milliliters of blood. The needle is removed from the vein, cotton soaked in alcohol is applied to the puncture site for 5-10 minutes.

The resulting material is transferred to a special test tube and sent to the laboratory for further research.

When erythremia is determined:

• The amount of iron in the blood.
• liver tests. Of the liver tests, the most informative is the determination of the level of alanine aminotransferase ( AlAT) and aspartate aminotransferase ( ASAT). These substances are contained in liver cells and are released into the blood in large quantities when they are destroyed.
• Bilirubin ( indirect fraction). When an erythrocyte is destroyed, the pigment bilirubin is released from it ( indirect or unrelated fraction). In the liver, this pigment quickly binds to glucuronic acid ( a direct bound fraction is formed) and is excreted from the body. Thus, the assessment of the indirect fraction of bilirubin provides information on the severity of the process of destruction of erythrocytes in the body.
• The amount of uric acid in the blood.

Changes in the biochemical analysis of blood in erythremia

Puncture of the bone marrow

The execution technique is quite simple, but, at the same time, painful for the patient ( carried out without anesthesia, as it can distort the data obtained) and is associated with certain risks ( puncture of the sternum and trauma to the lungs, heart, large blood vessels). Therefore, the procedure must experienced doctor and only under sterile operating conditions.

The site of the future puncture is thoroughly disinfected with a solution of alcohol or iodine, after which a special needle located in the center and at a right angle to the sternum pierces the skin and periosteum to a depth of 10-12 mm, falling into the bone cavity. A syringe is attached to the needle and 0.5 to 1 ml of bone marrow substance is drawn by pulling the piston, after which, without disconnecting the syringe, the needle is removed from the bone. The puncture site is closed with a sterile swab and sealed with a plaster.

The substance of the bone marrow coagulates faster than peripheral blood, so the resulting material is immediately transferred to a glass slide, on which the smear is stained with a special dye and fixed. Subsequently, the samples are examined under a microscope, and the data obtained are presented in the form of a table or chart, which is called a myelogram.

Microscopic examination evaluates:

• The number of cells in the bone marrow. In this case, their total number is first determined, and then a quantitative and percentage count of the cells of each of the germs - erythroid, platelet ( megakaryocytic) and leukocyte.
• The presence of foci of cancer cells.
• The presence of foci of proliferation of connective tissue ( signs of fibrosis).

Myelogram changes in erythremia

Laboratory markers

In the diagnosis of erythremia are used:

• determination of the total iron-binding capacity of serum;
• determination of the level of erythropoietin

In the nome, about 33% of the active centers of transferrin are bound to iron, the remaining 2/3 remain free. With a deficiency of this microelement, the liver produces a greater amount of transferrin, which allows fixing more iron. On the contrary, with an excess of iron in the body, it binds to a large number of free active centers of transferrin, as a result of which their number decreases.

The essence of the method is the gradual addition of a solution containing iron to the test blood until all the free active centers of transferrin are bound. Depending on the amount of iron required for complete saturation of transferrin, conclusions are drawn about the deficiency or excess of this microelement in the body.

To conduct a study, you need:

• exclude food intake 8 hours before blood donation;
• avoid alcohol and tobacco smoking 24 hours before blood donation;
• exclude physical activity 1 hour before blood donation.

The normal value of TIH can vary depending on the time of day, physical activity and food intake, but on average it is in the range from 45 to 77 µmol / l.

Erythremia is characterized by:

• At the initial stage - a moderate increase in TIBC as a result of increased formation of red blood cells in the bone marrow.
• In the erythremic stage a pronounced increase in TIBC due to iron deficiency in the body.
• In the anemic stage can be defined as a decrease in the FCL ( with severe bleeding) and its increase ( with fibrosis of the bone marrow and impaired formation of red blood cells).

In order to determine the level of erythropoietin, the method of enzyme immunoassay is used ( ELISA). The essence of the method is to identify the desired substance ( antigen) in the blood with the help of specific antibodies that interact only with this substance. The antigen in this case is erythropoietin.

The ELISA procedure consists of several successive stages. At the first stage, the blood to be examined is placed in the so-called “holes”, consisting of a special substance to which the desired antigen can be fixed ( erythropoietin).

A solution containing antibodies to erythropoietin is added to the wells. Antigen-antibody complexes are formed, which are firmly fixed on the surface of the well. A special marker is attached to the surface of antibodies in advance, which can change its color when interacting with certain substances ( enzymes).

At the second stage, the wells are washed with a special solution, as a result of which antibodies that are not connected to antigens are removed. After that, a special enzyme is added to the wells, which causes a color change of the markers fixed on the surface of the antibodies.

On the final stage using a special apparatus, the number of stained antigen-antibody complexes is counted, on the basis of which conclusions are drawn about the amount of erythropoietin in the test blood.

The norm of erythropoietin in plasma is 10 - 30 mIU / ml ( international milliunits in 1 milliliter). In the initial and erythremic stages this indicator reduced, since a large number of red blood cells inhibits the production of erythropoietin by the kidneys. In the terminal stage, with the development of anemia, the amount of erythropoietin in the blood significantly exceeds the norm.

Instrumental Research

For diagnostic purposes, apply:

• ultrasound procedure ( ultrasound) organs abdominal cavity;
• dopplerography.

The method is based on the ability of body tissues to reflect sound waves in different ways ( depending on their density and composition). Ultrasonic waves reflected from the surface of the organ under study are perceived by a special sensor, and after computer processing of the received signals, the monitor displays accurate data on the location, size and consistency of the organ.

Ultrasound of the internal organs with erythremia is characterized by:

• overflowing with blood of all internal organs;
• an increase in the size of the spleen and liver;
• foci of hyperechogenicity in the spleen and liver ( corresponding to fibrotic processes);
• the presence of infarcts in the spleen and liver ( cone-shaped hyperechoic zone).

The principle of the method is as follows - a special device emits ultrasonic waves, which, reflected from the blood in the vessel under study, are captured by a receiver located near the source of ultrasound.

The length and frequency of the reflected waves will be different depending on the direction of blood flow. After computer processing, the received information is displayed on the monitor. The areas of blood vessels where the blood flows in the direction from the ultrasound source are indicated in blue, and in the direction of the ultrasound source in red. This allows you to judge the blood supply to the organ under study.

With thrombosis of the vessels of the brain, spleen, liver and other organs, the blood flow in them may be reduced or completely absent ( depending on the degree of overlapping of the lumen of the vessel by a thrombus), which is confirmed by clinical manifestations of ischemia of these organs.

Treatment of erythremia

The main directions in the treatment of erythremia are :

• drug therapy of the underlying disease;
• improved blood flow;
• decrease in hemoglobin and hematocrit levels;
• elimination of iron deficiency;
• correction of urate diathesis;
• symptomatic therapy.

Medical therapy for erythremia

These drugs have a adverse reactions, the most dangerous of which is the development of acute leukemia. To reduce the risk of complications, treatment should be carried out only in a hospital, strictly observing the dosage and regimen of taking drugs, under constant monitoring of peripheral blood indicators - erythrocytes, platelets and leukocytes.

Indications for the use of cytostatics are:

• rapidly progressive increase in the number of blood cells ( erythrocytes, platelets and leukocytes);
• pronounced enlargement of the spleen and liver;
• frequent thrombotic complications ( strokes, heart attacks).

Medical treatment of erythremia

Improved blood flow

Methods for reducing blood viscosity

Decreased hemoglobin and hematocrit levels

Methods for lowering hemoglobin and hematocrit levels

Elimination of iron deficiency

Medicines that eliminate iron deficiency in the body

Correction of urate diathesis

Medicines that affect the metabolism of urate in the body

Symptomatic therapy

The main directions of symptomatic therapy are:

• Correction of high blood pressure - antihypertensive drugs ( Lisinopril, Amlodipine).
• Elimination of skin itching - antihistamines ( Periactin).
• Anemia correction - transfusion donated blood, washed erythrocytes, platelets.
• Improving the work of the heart with heart failure) – cardiac glycosides ( Strofantin, Korglikon).
• Prevention of the development of stomach ulcers - gastroprotectors ( Omeprazole, Almagel).
• Correction of increased destruction of blood cells in the spleen - surgical removal of an organ with the ineffectiveness of other methods of treatment).
• Consultations of other specialists - oncologist, neurologist, gastrologist, rheumatologist.

Prognosis for erythremia

The prognosis for erythremia is determined by:

• Early diagnosis - the earlier the disease is detected, the sooner its treatment will begin, and the prognosis will be more favorable.
• Adequate and timely therapy - with proper treatment, the symptoms of the disease can completely disappear.
• The level of erythrocytes, platelets and leukocytes in the blood - the higher it is during the course of the disease, the worse the prognosis.
• The body's response to treatment in some cases, erythremia progresses despite ongoing medical measures.
• The severity of fibrotic processes in the bone marrow - the more hematopoietic tissue remains in the bone marrow, the more favorable the outcome of the disease.
• Thrombotic complications - with thrombosis of the vessels of the brain, heart, liver, spleen, lungs and other organs, the prognosis is unfavorable.
• The rate of malignant transformation of the tumor - erythremia can turn into acute leukemia with a very severe course and death.

Erythremia is one of the types of blood diseases, which is characterized by the proliferation (growth) of red blood cells and a marked increase in the number of other cells (leukocytes, platelets). It is a chronic, often benign, disease that is quite rare. However, the degeneration of benign leukemia into malignant is possible.

Erythremia is a disease that affects about 4 out of 100 million people a year. It does not depend on gender, but is usually diagnosed in older and older people. Although cases have been reported in more than young age are often women.

Erythremia (ICD-10 code - C94.1) has a chronic course.

Blood cells and the mechanism of the disease

The mechanism of erythremia develops as follows.

Erythrocytes are red blood cells whose main function is to carry oxygen to all cells in our body. They are produced in the bone marrow, spleen and liver. Bone marrow is found in the tubular bones, vertebrae, ribs, skull bones, and sternum. 96% of red blood cells are filled with hemoglobin, which performs the respiratory function. Iron gives them a red tint. Red blood cells are formed with the help of a stem cell. Their distinctive ability is the ability to divide (reproduce), transforming into any other cell.

The bones also contain yellow bone marrow, which is represented by adipose tissue. It begins to produce blood elements only in extreme conditions, when other sources of hematopoiesis do not perform their functions.

Erythremia (ICD-10 - international classification of diseases, in which code C 94.1 is assigned to this ailment) is a disease whose pathogenesis has not yet been studied. For reasons still not fully understood by doctors, the body begins to intensively produce red blood cells, which no longer fit in the bloodstream, blood viscosity increases, and blood clots begin to form. Hypoxia builds up over time oxygen starvation). Cells do not receive proper nutrition, there is a failure in common system organism.

Some interesting facts about erythremia

1. Erythremia is the most benign of all leukemias. That is, she for a long time behaves passively and leads to complications rather late.
2. It may be asymptomatic and not report itself for many years.
3. In spite of elevated level platelets, patients are prone to heavy bleeding.
4. Erythremia has a family predisposition, therefore, if there is a patient in the family, the risk of its occurrence in one of the relatives increases.

Causes of erythremia

Like other erythremia, it has not yet been fully studied, and the reasons for its appearance have not been identified. However, the factors that are conducive to its appearance are voiced:

1. genetic predisposition.
2. Toxic substances that enter the body.
3. ionizing radiation.

genetic predisposition

It is currently unknown which gene mutations cause the appearance of erythremia, however, it has been established that the disease often recurs in the family in the next generations. The likelihood of developing the disease is increased significantly if a person suffers from:

• Down syndrome (violation of the shape of the face and neck, developmental delay);
• Klinefelter's syndrome (disproportionate figure and possible developmental delay);
• (short stature, pigmentation on the face and its disproportionate development, predisposition to cancer);
• Marfan's syndrome (disorders in the development of connective tissue).

The predisposition to erythremia is explained by the fact that the genetic cellular apparatus (including the blood apparatus) is unstable, so a person becomes susceptible to external negative effects - toxins, radiation.

Radiation

Even X-rays and gamma rays are partially absorbed by the body, affecting genetic cells. They may die or mutate.

However, the most powerful radiation receive people who treat cancer with chemotherapy, as well as those who were at the epicenter of explosions of power plants or atomic bombs.

Toxic Substances

When ingested, they can cause mutation of genetic cells. These substances are called chemical mutagens. After numerous studies, scientists found that patients with erythremia were in contact with these substances before the development of the disease. These substances include:

• benzene (found in gasoline and many chemical solvents);
• antibacterial drugs (in particular, Levomycetin);
• cytotoxic drugs (antineoplastic).

Stages of erythremia and their symptoms

Erythremia stage has the following: initial, expanded and terminal. Each of them has its own symptoms. The initial stage can last for decades without showing any serious symptoms. Patients usually attribute minor symptoms to other, less serious diseases. After all, even doctors often do not pay due attention to imperfect blood tests.

If there is initial erythremia, blood tests are moderately abnormal.

The initial stage is also characterized by fatigue, dizziness, tinnitus. The patient does not sleep well, feels cold in the extremities, swelling of the arms and legs appears. There is a decrease in mental performance. There are no external manifestations of the disease yet. Headache is not considered a specific sign of the disease, but it occurs at the initial stage due to poor circulation in the brain. For the same reason, vision, attention and intelligence are reduced.

With the diagnosis of "erythremia", the symptoms of the disease in the second stage have their own characteristics - the gums bleed, small hematomas become noticeable. May appear on the legs dark spots(symptoms of thrombosis) and even trophic ulcers. The organs in which red blood cells are formed are enlarged - the spleen and liver. Due to the development of the disease, the lymph nodes swell.

The second stage of erythremia can last about 10 years. The weight is noticeably reduced. The skin acquires a cherry tone (more often the arms and legs), the soft palate changes color, the hard palate retains its former shade. The patient is concerned about itching, which intensifies after bathing in warm or hot water. Swollen veins from an excess of blood become noticeable on the body, especially in the neck. The eyes seem to be bloodshot, because erythremia, the symptoms of which are quite obvious in the second stage, promotes blood flow to the vessels of the eyes.

Due to poor blood circulation in the capillaries, pain and burning appear in the fingers and toes. AT advanced cases cyatonic spots on them become noticeable.

Bone and epigastric pains take place. Joint pains of a gouty nature are associated with an excess of uric acid. In general, the work of the kidneys is disturbed, pyelonephritis is often diagnosed and urate stones are found in the kidneys.

The nervous system suffers. The patient is nervous, his mood is unstable, characterized by tearfulness and often changes.

The viscosity of the blood increases, which leads to the appearance of blood clots in a variety of vascular areas. There is also a risk of developing varicose veins.

Attention! Patients may also suffer from hypertension and open ulcers in the duodenum. This is associated with a decrease defensive forces organism and an increase in the number of Helicobacter pylori - it is this bacterium that causes an ulcer.

The third stage of erythremia is characterized by pallor of the skin, frequent fainting, weakness and lethargy. There are prolonged bleeding during minimal trauma, aplastic anemia due to a decrease in the level of hemoglobin in the blood.

The third stage of erythremia: symptoms and complications

Erythremia is a blood disease that becomes aggressive in the third stage. At this stage, the bone marrow cells undergo fibrosis. He can no longer produce red blood cells, so the norms of blood cells fall, sometimes to critical levels. Foci of softening appear in the brain, liver fibrosis begins. The gallbladder contains thick, viscous bile and pigment stones. The consequence of this is cirrhosis of the liver and blockage of blood vessels.

Previously, it was increased thrombus formation that caused the death of patients with erythremia. The walls of the vessels undergo changes, there is a blockage of the veins in the vessels of the brain, spleen, heart and legs. Occurs obliterating endarteritis - blockage of the vessels of the legs with the risk of their complete narrowing.

The kidneys are affected. Due to the growth of uric acid, patients suffer from articular pain of a gouty nature.

Attention! With erythremia, the legs and arms often change color. The patient is prone to bronchitis and colds.

The course of the disease

Erythremia is a slow disease. The disease comes into its own slowly, its onset is progressive and inconspicuous. Patients sometimes live for decades, not paying attention to minor symptoms. However, in more serious cases due to blood clots, death can occur within 4-5 years.

Along with erythremia, the spleen is enlarged. Allocate a form with cirrhosis of the liver and lesions diencephalon. During the course of the disease, allergic and infectious complications may be involved, sometimes patients do not tolerate certain groups of drugs, suffer from urticaria and other skin diseases. The course of the disease worsens comorbid conditions because the diagnosis is usually made in older people.

Posterythremic myelofibrosis is the degeneration of the disease from a benign course to a malignant one. The condition is natural in patients who have survived to this period. In this case, doctors admit that erythremia has acquired a tumor character.

It is extremely important to correctly diagnose erythremia and prescribe appropriate treatment. Diagnostics includes a number of studies.

Blood test for erythremia

With the diagnosis of "erythremia", a blood test, the indicators of which are very important for the diagnosis, is done first. First of all, this is a general blood test. It is deviations from the norm in it that become the first wake-up call. Increased number of red blood cells. Initially, it is uncritical, but with the development of the disease it increases more and more, and at the last stage it falls. Normally, the level of red blood cells in the blood in women is from 3.5 to 4.7, and in men - from 4 to 5.

If erythremia is diagnosed, a blood test, the indicators of which indicate an increase in red blood cells, also indicates a corresponding increase in hemoglobin. Hematocrit, indicating the ability of the blood to carry oxygen, is increased to 60-80%. The color indicator is not changed, but in the third stage it can be anything - normal, increased or decreased. Platelets and leukocytes are increased. Leukocytes grow several times, and sometimes even more. The production of eosinophils (sometimes together with basophils) is increased. Seriously Erythrocyte sedimentation rate is not higher than 2 mm/hour.

Alas, a general blood test, although important in initial diagnosis, is uninformative, and only on its basis the diagnosis is not made.

Other studies in erythremia

• Blood chemistry. Its main goal is to determine the amount of iron in the blood and the level of liver tests - AST and ALT. They are released from the liver when its cells are destroyed. Determining the amount of bilirubin indicates the severity of the process of destruction of erythrocytes.
• The puncture is taken with a needle, which is inserted into the skin, into the periosteum. The method indicates the state of hematopoietic cells in the bone marrow. The study indicates the number of cells in the bone marrow, the presence of cancer cells and fibrosis (overgrowth of connective tissue).
• With the diagnosis of chronic erythremia, laboratory markers and ultrasound of the abdominal cavity are also made. Ultrasound can detect overflow of organs with blood, enlargement of the liver and spleen, as well as foci of fibrosis in them.
• Dopplerography indicates the speed of blood movement and helps to identify blood clots.

Treatment of erythremia

It should be noted that erythremia often progresses slowly and has a benign course. First of all, doctors advise you to change your lifestyle - to visit more fresh air, walk, get positive emotions (endorphin therapy sometimes achieves amazing results). Foods that contain a large amount of iron and vitamin C should be avoided.

At the first stage of the disease, the main goal in the diagnosis of "erythremia" is to reduce blood counts to normal: hemoglobin - up to 150-160, and hematocrit - up to 45-46. It is also important to negate the complications caused by the disease - circulatory disorders, pain in the fingers, etc.

Bloodletting helps to normalize hematocrit with hemoglobin, which in this case is used to this day. However this procedure done as part of emergency care, because it stimulates the bone marrow and the function of thrombopoiesis (the process of formation of platelets). There is also a procedure called erythrocytepheresis, which removes red blood cells from the blood. However, the blood plasma remains.

Medical treatment

Cytostatics are antitumor drugs that are used for complications of erythremia - ulcers, thrombosis, circulatory disorders of the brain that occur in the second stage of the disease. Mielosan, Busulfan, hydroxyurea, Imiphos, radioactive phosphorus. The latter is considered effective due to the fact that it accumulates in the bones and depresses brain function.

Glucocorticosteroids are prescribed for hemolytic genesis. Prednisolone is especially popular. If the treatment does not give appropriate results, an operation is prescribed to remove the spleen.

To avoid iron deficiency, iron-containing preparations can be prescribed - Hemofer, Totema, Sorbifer.

Also, as necessary, the following groups of drugs are prescribed:

1. Antihistamines.
2. Reducing blood pressure.
3. Anticoagulants (blood thinners).
4. Hepatoprotectors.

Erythremia, which is often treated in a hospital, is a serious disease that causes many complications. It is important to identify it as early as possible and start therapy.

erythremia

What is Erythremia -

erythremia- chronic leukemia with a lesion at the cell level - a precursor of myelopoiesis with an unlimited growth of this cell, characteristic of a tumor, that retained the ability to differentiate into 4 sprouts, mainly in red. At certain stages of the disease, and sometimes from the very beginning, myeloid metaplasia in the spleen joins the proliferation of cells in the bone marrow.

What provokes / Causes of Erythremia:

Pathogenesis (what happens?) during Erythremia:

No specific cytogenetic abnormalities were found in erythremia.

Quantitative chromosome defects, structural aberrations are clonal in nature and are not found in lymphocytes. In patients treated with cytostatics, they are more common. According to the authors, patients with initially

detected violations of the chromosomal set are not predisposed to a more malignant course of the disease.

Although morphological, enzymatic and cytogenetic signs of lesions lymphatic system with erythremia is not available, the functional state of T-lymphocytes is changed: a reduced response to known mitogens and an increase in their spontaneous activity are found.

In the erythremic stage in the bone marrow, a complete disruption of the structure of the germs with displacement of fat is usually observed.

Besides classic version, changes of 3 more types can be observed: an increase in erythroid and megakaryocytic sprouts, an increase in erythroid and granulocytic sprouts; an increase in predominantly erythroid germ. Iron stores in the bone marrow are significantly reduced. The hematopoietic foothold is often dilated, and the fatty marrow may appear red, hematopoietic.

The spleen is congested with blood, contains areas of infarcts of various prescriptions, platelet aggregates, and often initial, moderate, or significant signs of myeloid metaplasia with localization in the sinuses. The follicular structure is usually preserved.

In the liver, along with plethora, there are foci of fibrosis, the connection of hepatic beams, sometimes myeloid metaplasia with localization in the sinusoids. Very thick bile and pigment stones are often seen in the gallbladder.

A frequent find is urate stones, pyelonephritis, wrinkled kidneys, significant pathology of their vessels.

In the anemic stage of the disease, there is a pronounced myeloid transformation of the spleen and liver, as well as their increase. The bone marrow is often fibrotic. At the same time, myeloid tissue can be both hyperplastic and reduced, the vessels of the bone marrow are sharply increased in number and structurally changed. In parenchymal organs, dystrophic and sclerotic changes are revealed. There are frequent manifestations of thrombotic syndrome or hemorrhagic diathesis.

The functional state of erythrocyte production, according to radiological studies, is sharply increased: the half-life is shortened radioactive iron injected into a vein, its utilization by the bone marrow is enhanced and the circulation is accelerated.

The average lifespan of platelets is often shortened, there is negative connection between their survival and the size of the spleen.

Symptoms of Erythremia:

The disease begins gradually. Reddening of the skin, weakness, heaviness in the head, enlarged spleen, arterial hypertension are increasing, and in half of the patients - excruciating skin itching after washing, washing, swimming. Sometimes the first manifestations of the disease are necrosis of the fingers, thrombosis of the larger arteries of the lower and upper extremities, thrombophlebitis, thrombotic stroke, myocardial or lung infarction, and especially acute burning pains in the fingertips, eliminated by acetylsalicylic acid for 1-3 days. Many patients, long before the diagnosis was established, had bleeding after tooth extraction, skin itching after a bath, and "good" red blood counts, which doctors did not attach due importance to.

In stage I, the duration of which is 5 years or more, there is a moderate increase in circulating blood, the spleen is not palpable. Moderate formation of red blood cells predominates in the blood at this stage. In the bone marrow, an increase in all hematopoietic sprouts. Vascular and visceral complications at this time are possible, but not frequent.

Isolation of the initial (I) stage of erythremia is conditional. In essence, this is a stage with asymptomatic manifestations, more characteristic of elderly patients. The spleen is usually not palpable, but its examination often reveals a slight increase. Thrombotic complications are also possible at this stage of the disease.

IIA stage of the process - erythremic - is developed, myeloid transformation of the spleen is uncharacteristic for it. The duration of this stage is 10-15 years or more. The volume of circulating blood is increased, the spleen is enlarged, and a little earlier, an increase in the liver is possible. Thrombosis of arterial and venous vessels, hemorrhagic complications at this stage are more common. A blood test indicates a "clean" erythrocythemia or erythrocythemia and thrombocytosis or panmyelosis and neutrophilia with a stab shift, an increase in the number of basophils. In the bone marrow, there is total three-growth hyperplasia with pronounced megakaryocytosis; reticulin and focal collagen myelofibrosis are possible.

Stage IIB also includes an erythremic, extensive process, but with myeloid metaplasia of the spleen. An increase in blood volume can be expressed to a greater or lesser extent, there is an increase in the liver and spleen. In the blood at this stage, there is an increase in erythrocytes, platelets with leukocytosis above 15 H 103 in 1 μl and a shift leukocyte formula to myelocytes, single erythrokaryocytes. In the bone marrow, as in stage IIA, an increase in the granulocytic germ may predominate, reticulin and focal collagen myelofibrosis is possible.

In the clinical picture, allergic complications and urate diathesis are often the leading ones.

At this stage, exhaustion of the patient, aggravated thrombotic complications and bleeding can be observed.

III stage of erythremia is called anemic. In the bone marrow, myelofibrosis can be expressed, myelopoiesis is preserved in some cases, and reduced in others. Myeloid transformation is observed in the enlarged spleen and liver. The outcome of erythremia at this stage may be acute leukemia, chronic myelogenous leukemia, hypoplastic state of hematopoiesis, and hematological changes that are difficult to classify.

Arterial hypertension that occurs with erythremia in 35-50% of cases is due to an increase in peripheral resistance in response to increased blood viscosity, the development of urate diathesis, chronic pyelonephritis, circulatory disorders in the renal parenchyma, thrombosis and sclerosis of the renal arteries.

Erythremia-specific pruritus associated with washing is observed in 50-55% of patients. In many patients, it becomes the main complaint, arises not only from contact with water, but also spontaneously, affects performance.

Frequent complications of the advanced stage of the disease are microcirculatory disorders with a clinic of erythromelalgia, transient disorders of cerebral and coronary circulation and hemorrhagic edema of the legs, as well as thrombosis of venous and arterial vessels and bleeding. Already at this stage, there may be violations of hemostasis, which often look like a latent thrombogenic danger, detected only in the laboratory and without clinical manifestations. At the same time, violations of hemostasis can be more pronounced, leading to local intravascular coagulation by the type of microthrombosis or to disseminated intravascular coagulation - DIC.

The mechanism of development of thrombotic complications of erythremia is to increase the mass of circulating erythrocytes, slow down blood flow and increase its viscosity. Their development is facilitated by thrombocytosis and qualitative disorders of platelets. In the blood plasma, circulating aggregates of platelets are often determined, which is the result of not only their quantitative increase, but also a violation of the functional properties of platelets.

Hemorrhagic complications of erythremia are completely eliminated in patients treated with bloodletting, when the hematocrit is normalized.

With the development of erythremia, iron deficiency is often observed, eliminating plethora. Clinical manifestations of iron deficiency - weakness, inflammation of the tongue, decreased resistance to infections, thinning of the nails - are more often observed in older people.

The development of the anemic stage is preceded by a certain dynamics of clinical and hemorrhagic data, in particular, an increase in the spleen, a gradual decrease in plethora, the appearance of a leukoerythroblastic picture of peripheral blood. Myelofibrosis gradually develops in the bone marrow, which may be accompanied by a change in type, cell proliferation, an increase in the pathology of the bone marrow vessels and inefficiency of hematopoiesis - the outcome of erythremia in secondary myelofibrosis.

There are other forms and variants of the course of the disease, in which an increase in the spleen due to myeloid transformation is detected from the very beginning. Exacerbations of the disease after treatment with cytostatics occur mainly with plethora and enlargement of the spleen. These are always pancytotic forms of the disease with a leukoerythroblastic blood picture, more severe than normal erythremia.

They differ from erythremia in early and pronounced extramedullary spread, a greater three-pronged growth orientation and reticulin myelofibrosis, and from idiopathic myelofibrosis- the presence of plethora and the duration of myeloproliferation, the absence of a tendency to the rapid completion of reticulin myelofibrosis.

At the same time, anemia that develops with erythremia may have a different mechanism of development, is not always associated with the progression of the process, and in many cases is successfully treated.

Anemia can be iron deficiency, caused by bleeding and bloodletting; hemodilution, associated with an increase in the volume of circulating plasma due to an increase in the spleen, hemolytic, caused by an increase in the function of the spleen. Finally, anemia in erythremia may be due to inefficient hematopoiesis. With the outcome of erythremia in acute leukemia or hypoplasia of hematopoiesis, anemia is observed, which is characteristic of these processes.

The frequency of the outcome of erythremia in acute leukemia is 1% in untreated and 11-15% in those treated with cytostatics (chlorbutine), acute myeloid leukemia and erythromyelosis develop more often. Harbingers of acute leukemia, sometimes occurring 2-3 years before its diagnosis, are non-infectious fever, unmotivated leukopenia, thrombosis or pancytopenia, sometimes dermatitis.

Posterythremic myelofibrosis- the result of the natural evolution of the disease. It is observed in every patient with erythremia, surviving to this period. The difference in its hematological manifestations and course is striking - from benign, with hematological compensation, to malignant, with rapid anemia, depression of granulo- and thrombopoiesis, sometimes with low-percentage blastemia. In these cases, it is probably necessary to assume a tumor progression of the disease, before the manifestations of which in the form of a blast crisis, months and years may pass.

Diagnosis of Erythremia:

Diagnosis of erythremia is complicated by the fact that it is not the only cause of erythrocytosis.

There are the following types of red blood.

1. Erythremia.
2. Secondary absolute erythrocytosis (due to increased formation of erythropoietins).
3. With generalized tissue hypoxia (hypoxic, compensatory):

1) with arterial hypoxemia: "altitude" disease, chronic obstructive pulmonary disease, congenital "blue" heart defects, arteriovenous fistulas, carboxyhemoglobinemia (mainly due to tobacco smoking);

2) without arterial hypoxemia: hemoglobinopathies with increased affinity for oxygen, deficiency of 2,3-diphosphoglycerate in erythrocytes.

For tumors: kidney cancer, cerebellar hemangioblastoma, Hippel-Lindau syndrome, hepatoma, uterine fibroids, tumors of the cortical and medulla of the adrenal glands, pituitary adenoma and cyst, masculinizing ovarian tumors.

With local ischemia of the kidneys (dysregulatory): kidney cysts (solitary and multiple), hydronephrosis, rejection of the kidney transplant, stenosis of the renal arteries.

1. Cobalt (mainly experimental).
2. Secondary relative, hemoconcentration erythrocytosis: stress erythrocytosis, Geisbeck's syndrome, pseudopolycythemia.
3. primary erythrocytosis.

Erythremia is diagnosed according to certain standardized criteria. Erythremia can be suspected by an increase in red blood and hematocrit in peripheral blood: for men, more than 5.7 H 106 erythrocytes in 1 μl, HB more than 177 g/l, Ht 52%; for women more than 5.2 h 106 erythrocytes in 1 µl.

The criteria for diagnosing erythremia are as follows.

1. An increase in the mass of circulating red blood cells: for men - more than 36 ml / kg, for women - more than 32 ml / kg.

1. Normal saturation of arterial blood with oxygen (more than 92%).
2. Enlargement of the spleen.
3. Leukocytosis more than 12 H 103 in 1 µl (in the absence of infections and intoxications).
4. Thrombocytosis more than 4 hours 105 in 1 µl (in the absence of bleeding).
5. An increase in the content of alkaline phosphatase of neutrophils (in the absence of infections and intoxications).

7. Increase in unsaturated vitamin B 12-binding capacity of blood serum.

The diagnosis is reliable with any 3 positive signs.

With plethora, enlargement of the spleen, leukocytosis and thrombocytosis, the diagnosis of erythremia is not difficult, but even in these cases, it is necessary to study the ilium to confirm the diagnosis and comparative diagnosis with other myeloproliferative diseases.

Diagnostic problems arise in relation to purely erythrocytemic forms of polycythemia without enlargement of the spleen, which can turn out to be both erythremia and erythrocytosis: about 30% of patients with erythremia do not have leukocytosis and thrombocytosis at diagnosis.

Comparative diagnosis requires radiological measurement of the mass of circulating erythrocytes, and sometimes the volume of circulating plasma using serum albumin.

When a normal mass of circulating erythrocytes and a reduced plasma volume are detected, a relative increase in erythrocytes is diagnosed.

Relative erythrocytosis should be assumed when increased rates red blood patients have the usual color of the skin and mucous membranes.

With an increase in the mass of circulating erythrocytes, a comparative diagnosis is made between erythremia and absolute erythrocytosis. At smoking study the content of carboxyhemoglobin is carried out in the morning, afternoon and evening, as well as 5 days after smoking cessation.

With the exclusion of hypoxic erythrocytosis, the object of study should be the kidneys, and then other organs and systems, the diseases of which are accompanied by erythrocytosis.

Histological examination of the ilium allows the doctor to establish correct diagnosis in 90% of cases. Occasionally, there are no changes in the bone marrow with erythremia, and then the doctor can make a diagnosis of erythremia only with a convincing clinical and hematological picture.

For comparative diagnosis of erythremia and erythrocytosis, erythropoietins are examined, the number of which is reduced in erythremia, and increased in erythrocytosis.

The morphological and functional characteristics of blood cells should be taken into account. Erythremia is confirmed by large forms of platelets and a violation of their aggregation properties; increase in the number of neutrophils more than 7 h 103 in 1 µl; an increase in the content of alkaline phosphatase in them; detection high content on the membrane of neutrophils receptors for IgG; increase in the content of lysozyme; increase

absolute number of basophils (acrylic blue staining) more than 65/µl; an increase in the content of gnetamine in the blood and urine (secretion product of basophils).

Patients in whom the causes of polycythemia could not be identified should be included in the group of patients with unclassified polycythemia. Cytostatic treatment is not indicated for such patients.

Erythremia Treatment:

The task of treatment- normalization of the amount of hemoglobin to 140-150 g / l (85-90 IU) and hematocrit (46-47%), since it is at this time that the risk of vascular complications is sharply reduced. Bloodletting is prescribed in 500 ml every other day in the hospital and after 2 days in outpatient treatment. Instead of bloodletting, it is better to carry out erythrocytapheresis. The number of phlebotomies is determined by the achievement of normal red blood counts.

In elderly patients, or those with concomitant diseases of the cardiovascular system, or who do not tolerate bloodletting, no more than 350 ml of blood is removed once, and the intervals between bloodletting are somewhat lengthened. To facilitate bloodletting and prevent thrombotic complications on the eve and on the day of the procedure or during the entire period of bloodletting, as well as 1-2 weeks after the end of treatment, antiplatelet therapy should be prescribed - acetylsalicylic acid at 0.5-1 g / day and chimes at 150-200 mg/day at the same time. Additionally, immediately before bloodletting, the introduction of 400 ml of rheopolyglucin is recommended.

With contraindications to the use of acetylsalicylic acid, the doctor prescribes chimes, papaverine or drugs nicotinic acid. At the end of treatment, the condition of patients and the blood picture is monitored every 6-8 weeks.

Indications for the appointment of cytostatics are erythremia with leukocytosis, thrombocytosis and enlargement of the spleen, skin itching, visceral and vascular complications, serious condition patient, as well as the lack of effectiveness of previous treatment with bloodletting, the need for their frequent repetition, poor tolerance and complication of both stable thrombocytosis and clinically manifested iron deficiency. In the latter case, against the background of treatment with cytostatics, iron replacement therapy is performed. The advanced age of patients (over 50 years), the inability to organize bloodletting therapy expand the indications for treatment with cytostatics.

Cytostatic therapy is usually combined with phlebotomy given until hematocrit and hemoglobin normalize from the very beginning of cytostatic therapy.

Hematological monitoring of the course of treatment is carried out weekly, and by the end of treatment - every 5 days.

Urate diathesis is an indication for the appointment of milurite (allopurinol) in a daily dose of 0.3 to 1 g. The drug reduces the synthesis of uric acid from hypoxanthine, the content of which increases due to cellular

hypercatabolism. In the treatment of cytostatics, the drug is prescribed prophylactically in a daily dose of 200 to 500 mg or more.

Microcirculatory disorders and, in particular, erythromelalgia (attacks of sudden burning pains, mainly in the extremities with local redness and swelling of the skin), caused mainly by an aggregation block of arterial blood flow at the y-level of capillaries and small arteries, are successfully treated with acetylsalicylic acid, 0.31 g per day. day. The effectiveness of one chimes in erythromelalgia is much lower.

It should be noted that appeared in connection with the widespread use of acetylsalicylic acid gastrointestinal bleeding, including prolonged and representing a real danger. Prolonged nasal and gingival bleeding is possible.

This complication of treatment is caused by both unrecognized ulcerative lesions gastrointestinal tract, characteristic of erythremia and asymptomatic, and the initial functional defectiveness of platelets, aggravated by acetylsalicylic acid.

Acute vascular thrombosis- an indication for the appointment of not only platelet deaggregants, but also heparin, fresh frozen plasma transfusions.

When treating in the anemic phase, the mechanism of development of anemia, thrombocytopenia and other symptoms is taken into account. With anemia caused by iron or folic acid deficiency, appropriate replacement therapy is prescribed. Treatment of hemodilution anemia should be aimed at shrinking the spleen with radiotherapy, cytostatics and prednisolone. Anemia caused by insufficient production of red blood cells is preferably treated with androgens or anabolic steroids. Prednisolone is prescribed mainly for suspected autoimmune origin of anemia and thrombocytopenia, as well as to reduce the spleen.

2 treatment regimens are used:

1) the appointment of a high dose of prednisolone - 90-120 mg / day for 2 weeks, followed by a transition to medium and small doses with an effect and drug withdrawal if ineffective;

2) the appointment from the very beginning of average daily doses (20-30 mg), and then small doses (15-10 mg) for 2-3 months with the obligatory withdrawal of the drug. In many cases, there is a clear positive effect of steroid therapy, although the mechanism of its action is not completely clear.

For outcomes in acute leukemia, polychemotherapy is used, taking into account the histochemical variant, and for outcomes in typical and atypical myeloid leukemia, myelosan and myelobromol, hydroxyurea, but with little effect. With posterythremic myelofibrosis, increasing leukocytosis and progression of splenomegaly, short courses of therapy with myelobromol (250 mg / day) or myelosan (4-2 mg / day for 2-3 weeks) are advisable.

In anemic and thrombocytopenic syndromes, glucocorticosteroids are used, often in combination with cytostatics (in small doses) if an enlarged spleen is suspected. For the same purpose, you can apply y-therapy to the spleen area in a course dose of 5 Gy, sometimes a little more, if the number of platelets allows. A positive effect of small doses of prednisolone (15-20 mg / day), prescribed for 2-3 months, on the size of the spleen, general manifestations of the disease and the blood picture has been noted, but it is limited to the period of treatment and the immediate time after its cancellation.

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Other diseases from the group Diseases of the blood, hematopoietic organs and individual disorders involving the immune mechanism:

Erythremia is classified as a neoplastic disease, as it occurs with a pathological increase in red blood cells. In some cases, there is a parallel increase in the concentration of white blood cells and platelets. Often, the disease is not considered oncological, and the increase in the number of red blood cells is benign. Patients reside long life without any complications in the diagnosis of erythremia, so that the disease does not have a serious negative effect on the body.

Often patients have a question: "What is erythremia?". The answer is unequivocal - this is a disease of the blood system, which is characterized by a steady and significant increase in the number of formed elements, predominantly red blood cells, or erythrocytes.

The disease belongs to the group of tumors, but is not considered malignant. However, it should be noted that the prognosis for the patient's life is conditionally favorable, since in some cases progressive complications are observed, which in a relatively short time lead to the death of the patient.

Most often, erythremia is diagnosed in elderly patients, predominantly female. However, according to the latest statistics, the disease is "younger" - now the pathology is recorded in patients of middle and young age. The disease is extremely rare, about 5 people out of 100 million suffer from a blood disease.

Classification of erythremia

According to the international classification of diseases, erythremia belongs to the group of leukemias. There are 2 main forms of the course of the disease - acute and chronic. In addition, there are a number of signs by which the pathological condition can be divided into several groups.

According to the course of the disease, such are distinguished.

1. Polycythemia vera is an increase in the concentration of red blood cells due to a constant increase in the number of cells in a certain volume of blood.
2. False - the concentration of red blood cells increases not due to an increase in their number, but due to a decrease in the volume of circulating blood (dehydration, blood loss).

The cause of the pathology is distinguished:

• primary;
• secondary.

Causes of erythremia

The causes of the pathological condition are not fully understood, but doctors identify several main precursors of erythremia.

1. Genetic predisposition of the patient to diseases of the hematopoietic organs. Certain mutations at the gene level, which occur both during gametogenesis in parents and at the stage of embryonic development, have a negative impact on the correct laying of organs and their subsequent functioning. In addition, based on medical statistics, erythremia is more common in patients with serious genetic, chromosomal and genomic mutations, such as Marfan, Down and Klinefelter syndrome.
2. Exposure to ionizing radiation. Prolonged exposure to radiation substances also affects the genetic apparatus of most human cells, causing spontaneous mutations and damage to the structure of the DNA molecule. Thus, professions associated with constant, long-term contact with radioactive substances become the cause of the development of true polycythemia.
3. Poisoning with toxic substances. Benzene, cyclophosphamide, azathioprine and other similar toxic compounds can cause mutations at the cellular level. Constant contact causes serious damage to the genetic apparatus of the cell, spontaneous and irreversible changes, which subsequently become the cause of the development of a pathological condition.

Pathogenesis

The development and course of the disease is directly related to the occurrence of disturbances in the formation of formed elements in the red bone marrow, the main organ of hematopoiesis in adult patients. The development of blood cells from a pluripotent predecessor occurs indefinitely, and the descendants acquire a specific genotype.

As a result, the process of increasing the number and maturation of these formed elements of the erythrocyte series does not depend on the hormone-like substance erythropoietin produced by the kidneys with an insufficient level of oxygenation of the body.

Similar erythroid colonies of bone marrow cells are the main differential sign erythremia, which makes it possible to distinguish this pathology from the usual increase in the number of red blood cells that occurs after blood loss or in conditions of increased physical exertion.

In addition, there are a number of changes that affect the entire body of a sick person:

• dystrophic processes are revealed in parenchymal organs;
• red bone marrow is often fibrotic, accumulation of adipose tissue is found;
• the vessels of the bone marrow change their own structure, their number increases dramatically;
• the supply of iron decreases;
• hemoglobin is synthesized in large quantities, oxygen does not reach the organs and tissues in the volume necessary for oxidation due to dysfunction of the molecules;
• increases the level of uric acid in the blood;
• the life expectancy of blood cells - platelets, leukocytes, erythrocytes - is significantly reduced.

Due to the increase in the number of old blood cells, the spleen and liver increase in size. This serves as another non-specific diagnostic sign of erythremia.

The clinical picture of erythremia

Symptoms of erythremia appear gradually. Due to an increase in the number of red blood cells that cannot fully perform their own functions, patients experience:

• redness of the skin, especially the face;
• patients complain about typical symptoms hypoxia: headache, fatigue, dizziness;
• significantly enlarged liver - hepatomegaly;
• the size of the spleen significantly exceeds normal values;
• after prolonged contact with water, unbearable itching occurs;
• the duration of bleeding increases significantly;
• decreased visual, auditory sensitivity;
• redness of the conjunctiva of the eyes due to an increase in the number of red blood cells;
• varicose veins of the deep veins of the lower extremities;
• violation of the functioning of the immune system, an increase in infectious, viral diseases;
• the appearance of necrotic changes in the toes and hands is possible;
• due to an increase in blood viscosity, blood clots form, the consequences of which can be myocardial infarction;
• aggravation of the inflammatory process in the gastrointestinal tract is possible, perforating ulcers of the stomach or duodenum often appear.

In chronic erythremia, all such signs appear relatively slowly and are less pronounced than in the acute form of pathology. However, changes in the body must be immediately described to the attending physician in order to avoid serious complications that can lead to death.

Erythremia in children is often caused by another disease and is a symptom, relative or false pathology of the hematopoietic organs. This form of flow is called secondary erythremia, the symptoms and treatment will depend on the underlying disease.

Stages of erythremia

This pathology The blood system proceeds in several stages. In total, doctors distinguish 3 main stages of the disease.

initial stage

This period can take from 2-3 months to 5-6 years. The patient during this period of time does not notice any significant changes in own body. It is detected only with a general blood test (an increase in the concentration of red blood cells and hemoglobin levels), but not every doctor undertakes to differentiate the disease.

erythremic stage

At this stage, the first manifestations of pathology appear. Due to the increase in the amount of blood, all organs are filled, pressure rises, redness of the skin and mucous membranes of the mouth, nose, and eyes is observed. Plasma viscosity is relatively increased, which causes the formation of stagnation, thrombosis of arterial and venous vessels occurs.

Blood flow to the spleen and liver gradually increases, which provokes a significant excess in the size of parenchymal organs. Visual impairment and hearing loss may occur. Bleeding is already relatively difficult to stop. Pathologically altered erythrocytes do not tolerate the required amount of oxygen, which disrupts the work of some structures, mainly skeletal muscles.

anemic stage

At this stage of the development of the disease, irreversible changes occur in the red bone marrow. The hematopoietic organ undergoes fibrosis, the sites are replaced by adipose tissue. Thus, the production of blood cells gradually decreases, which leads to anemia. Due to a significant decrease in the number of erythrocytes, leukocytes and platelets, the spleen and liver take on an additional load - foci of hematopoiesis appear in the organs, which should compensate for the lack of blood cells.

The acute and chronic form of the disease at the terminal stage is characterized by prolonged, large-scale bleeding - often doctors fail to stop them in time. The patient is worried about headache, weakness and other symptoms of hypoxia, which are especially dangerous for a child, a pregnant woman.

Which doctors should be consulted

Erythremia is a serious blood disease that can result in unpleasant consequences for the patient. For early detection and treatment similar pathology You will need to be examined by several doctors. First you need to find out if there is a danger of developing the disease: occupational risks, genetic predisposition, recent contact with radioactive or toxic substances.

If there is still a cause for concern, then you need to contact the clinic at the place of residence and go through the initial stages of the examination with the attending physician. If the doctor has difficulties in making a diagnosis, he will appoint consultations with other specialists - an oncologist, a hematologist.

Diagnosis of erythremia

Laboratory diagnosis of this pathology includes.

1. Indicators of a blood test (general and biochemical, hematocrit) are required in order to assess the increase in the mass of circulating formed elements, to identify an increase in blood viscosity. An increase in the number of erythrocytes, but fewer platelets and leukocytes in a quantitative ratio.
2. Bone marrow puncture for follow-up microscopic examination biopsy. This technique allows you to identify changes at the level of the precursor cell of all rows of blood cells.
3. Ultrasound examination of the liver and spleen.

Such an examination can be supplemented depending on the stage of the disease, for example, when identifying the causes of anemia caused by an unidentified pathology of the hematopoietic organs.

Treatment of erythremia

Treatment of erythremia begins immediately after diagnosis. The therapy has the following goals:

• stabilization of blood circulation processes;
• elimination of the main cause of the pathology;
• symptomatic treatment;
• restoration of an acceptable iron concentration.

Nutrition and diet

The patient needs to consume a lot of foods containing large amounts of iron, as well as vitamins of group B, C, which are involved in the processes of hemoglobin synthesis:

• liver;
• buckwheat, brown rice, oatmeal;
• eggplant, tomatoes, greens;
• dairy products, cheeses;
• fresh vegetables and fruits, mostly red, orange and yellow.

Medical treatment

At the second stage of the disease, when erythremia is detected without problems, doctors recommend the use of cytostatic drugs. Although the pathology is not considered malignant, patients are required to undergo non-specific treatment in an oncology dispensary.

Data medicines used for violations of bone marrow cell proliferation, however, any cytostatic drug is not recommended for a long time due to a significant list of side effects from the hematopoietic organs. In addition, acetylsalicylic acid is included in the therapy to reduce inflammation.

Other techniques

In addition to drug therapy, some types of alternative treatment such as hirudotherapy or bloodletting. However, these methods are extremely dangerous for the patient's life, especially at the terminal stage of the disease, when there are few platelets.

Bloodletting is prescribed 500 ml per procedure, which is carried out under the close attention of the attending physician.

Prevention and prognosis

It is possible to talk about the prevention of the disease only when the patient does not have a genetic predisposition to pathology. In addition, it is required to minimize contacts with toxic and radioactive substances in order to avoid the appearance of mutations in the cell genome.

The prognosis for erythremia is often conditionally positive, since the disease is treatable. In chronic polycythemia, life expectancy is 5-25 years after the detection of pathology, provided there are no serious complications.

Possible Complications

The most serious complications in this disease are acute thrombosis, deterioration in the sensitivity of the auditory and visual analyzer, multiple organ failure and death of the patient. A patient who lives to a ripe old age may have serious problems with all organs, from the musculoskeletal system to the central nervous system.

Tumor disease, one of the varieties of chronic leukemia, often benign in nature, is called erythremia (aka Wakez-Osler disease, polycythemia vera, erythrocythemia).

What is erythremia

With erythremia, erythrocyte proliferation (growth) occurs and the number of other cells increases greatly (pancytosis). There is also an increase in hemoglobin.

Is erythremia an oncology, is it cancer or not? Most often, erythremia is benign, with a long course, but degeneration can occur. benign form disease into malignant, followed by death.

Erythremia is a fairly rare disease. It is diagnosed in about 4 out of 100 million people a year. There is no dependence of the disease on the person's gender, but it usually develops in patients over 50. At a young age, cases of the disease are more often diagnosed in women. Erythremia (ICB code 10 - C 94.1) is characterized by a chronic form of the course.

Note! Erythremia is considered one of the most benign blood diseases. Death often occurs due to the progression of various complications.

The disease is asymptomatic for a long time. Patients with this diagnosis have a tendency to profuse bleeding (although the level of platelets, which are responsible for stopping bleeding, is increased).

There are two types of erythremia: acute form (erythroleukemia, erythromyelosis, erythroleukemia) and chronic. Depending on the progression, they are divided into true and false. The first form is characterized by a persistent increase in the number of blood cells, which is extremely rare in children. The peculiarity of the second type is that the level of erythrocyte mass is normal, but the plasma volume is slowly decreasing. Depending on the pathogenesis, the true form is divided into primary and secondary (erythrocytosis).

How does erythremia develop?

Erythremia develops as follows. Red blood cells (their function is to deliver oxygen to all cells of the human body) begin to be intensively produced so that they cannot fit in the bloodstream. When these red blood cells overflow bloodstream- increased blood viscosity and thrombosis. Hypoxia increases, cells receive less nutrition, and failure begins in the entire work of the body.

Where are erythrocytes formed in the human body? Erythrocytes are produced in the red bone marrow, liver, and spleen. A distinctive ability of erythrocytes is the ability to divide, transforming into another cell.

The norm for the content of hemoglobin in the blood is different and depends on age and gender (in children and older people, its amount is lower). The normal content of red blood cells (normocytosis) in 1 liter of blood is:

• for men - 4.0 - 5.0 x 10 12;
• in women - 3.5 - 4.7 x 10 12.

The cytoplasm of an erythrocyte is almost 100% occupied by hemoglobin containing an iron atom. Hemoglobin gives the red color to red blood cells, is responsible for delivering oxygen to all organs and removing carbon dioxide.

The formation of erythrocytes goes on constantly and continuously, starting from prenatal development(in the third week of embryo formation) and until the end of life.

As a result of mutations, a pathological clone of a cell is formed that has similar abilities for modification (it can become an erythrocyte, platelet or leukocyte), but is not controlled by the regulatory systems of the body that maintain the cellular composition of the blood. The mutant cell begins to multiply, and the result of this is the appearance of absolutely normal red blood cells in the blood.

So there are 2 different types of cells - normal and mutant. As a result, the number of mutated cells in the blood grows, exceeding the needs of the body. This inhibits the excretion of erythropoietin by the kidneys and leads to a decrease in its effect on the normal process of erythropoiesis, but does not affect the tumor cell. As the disease progresses, the number of mutant cells grows, they crowd out normal cells. There comes a time when all the red blood cells of the body arise from a tumor cell.

With erythremia, the maximum of mutant cells turns into nuclear-charged erythrocytes (erythrocytes), but a certain part of them develops with the formation of platelets or leukocytes. This explains the increase in the number of not only red blood cells, but also other cells. Over time, the level of platelets and white blood cells derived from the cancer cell increases. There is a process of slugging - blurring of the boundaries between red blood cells. But the shell of erythrocytes is preserved, and slugging is a pronounced aggregation - crowding of erythrocytes. Aggregation leads to a sharp increase in blood viscosity and a decrease in its fluidity.

Causes of erythremia

There are some factors that predispose to the occurrence of erythremia:

• genetic predisposition. If there is a patient in the family, the risk of its development in one of the relatives increases. The risk of the disease also increases if a person has: Down syndrome, Klinefelter's syndrome, Bloom's syndrome, Marfan's syndrome. The predisposition to erythremia is explained by the instability of the genetic cellular apparatus, because of this, a person becomes susceptible to negative external influence– toxins, radiation;
• ionizing radiation. X-rays and gamma rays are partly absorbed by the body, affecting genetic cells. But people who are treated for oncology with chemotherapy, and those who were at the epicenter of explosions of power plants, atomic bombs, are subject to the strongest radiation;
• toxic substances found in the body. When ingested, they can contribute to the mutation of genetic cells, such substances are called chemical mutagens. These include: cytostatic drugs (antineoplastic - "Azathioprine", "Cyclophosphan"), antibacterial ("Levomycetin"), benzene.

Stages of the disease and symptoms of erythremia

Erythremia is a long-term disease. Its beginning is inconspicuous. Patients often live for decades without considering minor symptoms. But in more serious cases, due to the formation of blood clots, death can occur in 4-5 years. Along with the development of erythremia, the spleen grows. Allocate a form of the disease with cirrhosis of the liver and damage to the diencephalon. Allergic and infectious complications can be involved in the disease, often patients do not tolerate certain medications, suffer from hives and other skin diseases.

The course of the disease can be complicated by other diseases due to the fact that older people are more likely to suffer from the underlying disease. Initially, erythremia does not manifest itself and has practically no effect on the body as a whole and the circulatory system. As it develops, complications and pathological conditions may occur.

During erythremia, the following stages are distinguished:

• initial;
• erythremic;
• anemic (terminal).

Each of them has its own symptoms.

initial stage

The initial stage of erythremia lasts from several months to decades, may not appear. Blood tests have small deviations from the norm. This stage is characterized by fatigue, tinnitus, dizziness. The patient does not sleep well, feels cold in the extremities, swelling and pain in the arms and legs. There may be reddening of the skin (erythrosis) and mucous membranes - the area of ​​​​the head, limbs, oral mucosa, and eye membranes.

At this stage of the disease, this symptom is not so pronounced, so it can be taken as the norm. There may be a decrease in mental abilities. Headache is not a specific symptom of the disease, but is present at the initial stage due to poor blood circulation in the brain. Because of this, vision falls, intelligence and attention decrease.

erythremic stage

In the second stage of the disease, the number of red blood cells that arise from the abnormal cell increases. As a result of mutations, the mutated cell begins to change into platelets and leukocytes, increasing their number in the blood. Vessels and internal organs overflow with blood. The blood itself becomes more viscous, the speed of its passage through the vessels decreases, and this contributes to the appearance of platelets in the vascular bed, platelet plugs are formed that clog the gaps of small vessels, interfering with the flow of blood through them. There is a risk of developing varicose veins.

Erythremia at stage 2 has its own symptoms - the gums bleed, small hematomas are visible. Obvious symptoms of thrombosis appear on the lower leg - dark spots, lymph nodes swell and trophic ulcers appear. The organs where red blood cells are formed are enlarged - the spleen (splenomegaly) and the liver (hepatomegaly). The functioning of the kidneys is impaired, pyelonephritis is often diagnosed and urate stones are found in the kidneys.

Stage 2 erythremia can last about 10 years. There is itching, aggravated after contact with warm (hot) water. The eyes appear to be filled with blood, this is due to the fact that erythremia promotes blood flow to the eye vessels. Soft sky changes color strongly, solid - remains with the same color - this is the development of Cooperman's symptom.

Bone and epigastric pains are felt. Pain in the joints of a gouty nature occurs due to an excess of uric acid. There may be attacks of acute, burning pain (erythromelalgia) in the area of ​​the tip of the nose, earlobes, tips of the toes and hands, which occurs due to impaired blood circulation in the peripheral vessels. The nervous system suffers, the patient becomes nervous, his mood is changeable. Symptoms of iron deficiency occur:

• lack of appetite;
• dry skin and mucous membranes;
• cracks in the corners of the mouth;
• indigestion;
• decreased immunity;
• violation of taste and olfactory functions.

anemic stage

With the development of the disease in the bone marrow, fibrosis occurs - the replacement of hematopoietic cells with fibrous tissue. The hematopoietic function of the bone marrow slowly decreases, which leads to a decrease in the number of erythrocytes, platelets, and leukocytes in the blood. As a result, extramedullary foci of blood formation appear in the liver and spleen. Cirrhosis of the liver and blockage of blood vessels are a consequence of this. The walls of the vessels are modified, there is a clogging of the veins in the vessels of the brain, spleen, heart.

Obliterating endarteritis appears - clogging of the vessels of the legs with an increasing risk of their complete narrowing. The kidneys are affected. The main manifestations in the third stage of erythremia are pallor of the skin, frequent fainting, weakness and lethargy. There may be prolonged bleeding even due to minimal injuries, aplastic anemia due to a decrease in the level of hemoglobin in the blood. In the third stage, erythremia becomes aggressive.

On a note! With erythremia, the skin of the legs and arms can change color. The patient is predisposed to bronchitis and colds.

Diagnosis of erythremia

For diagnosing erythremia, the most important CBC is a complete blood count. Deviations of these laboratory parameters relative to the norm become the first sign of the disease. At the very beginning, blood counts do not differ much from the norm, but with the development of the disease they increase, and at the last stage they fall.

If erythremia is diagnosed, blood counts indicate an increase in red blood cells and hemoglobin. Hematocrit, indicating the ability of the blood to carry oxygen, rises to 60-80%. Platelets and leukocytes are increased. Only on the basis of KLA the diagnosis is not made. In addition to a general blood test, they carry out:

• blood chemistry. It detects the iron content in the blood and the value of liver tests (AST and ALT). The level of bilirubin indicates the severity of the process of destruction of erythrocyte cells;
• bone marrow puncture. This analysis shows the state of hematopoietic cells in the bone marrow - their number, the presence of tumor cells and fibrosis;
• Abdominal ultrasound. This examination helps to see the overflow of organs with blood, the growth of the liver and spleen, foci of fibrosis;
• dopplerography. Detects the presence of blood clots and shows the speed of blood movement.

Also, when diagnosing erythremia, the level of erythropoietin is determined. This study determines the state of the hematopoietic system and indicates the level of red blood cells in the blood.

Pregnancy with erythremia

When a woman is in position, there is no need to fear that she will pass on the pathology to the child. The heredity of the disease is still not fully understood. If the pregnancy was asymptomatic, erythremia will not have any effect on the baby.

Treatment of erythremia

Polycythemia progresses slowly. In the first stage of polycythemia, the main goal of treatment is to reduce blood counts to normal: hemoglobin and hematocrit of the erythrocyte mass. It is also important to minimize the complications caused by erythremia. Bloodletting is used to normalize hematocrit with hemoglobin. There is a procedure called erythrocytepheresis, which means the purification of the blood from red blood cells. The blood plasma is preserved.

Drug treatment includes the use of cytostatics (drugs of antineoplastic nature) that help with complications (thrombosis, ulcers, circulatory disorders of the brain). This includes Mielosan, Busulfa, Imiphos, hydroxyurea, radioactive phosphorus.

With hemolytic anemia of autoimmune genesis, glucocorticosteroids are used - Prednisolone. If such therapy does not work, an operation is performed to remove the spleen.

In order to avoid iron deficiency, iron-containing preparations are prescribed - Maltofer, Hemofer, Sorbifer, Totema, Ferrum Lek.

If necessary, the following groups of drugs are prescribed:

• lowering blood pressure - "Lizinopril", "Amlodipine";
• antihistamines - "Periaktin";
• blood thinners (anticoagulants) - Aspirin, Curantil (Dipyridamole), Heparin;
• improving the work of the heart - "Korglikon", "Strophanthin";
• for the prevention of the development of stomach ulcers - gastroprotectors - "Almagel", "Omeprazole".

Diet for erythremia and folk remedies

To combat the disease, the patient should follow a vegetable and sour-milk diet. Proper nutrition includes the use of foods such as:

• vegetables - raw, boiled, stewed;
• kefir, cottage cheese, milk, yogurt, sourdough, curdled milk, fermented baked milk, sour cream;
• eggs;
• tofu, brown rice dishes;
• whole wheat bread;
• greens (spinach, dill, sorrel, parsley);
• almond;
• dried apricot grapes;
• tea (preferably green).

Red-colored vegetables and fruits and juices from them, soda, sweets, fast food, smoked meats are contraindicated. The amount of meat consumed should be limited.

To prevent the appearance of blood clots in stage 2 of the disease, they drink juice prepared from horse chestnut flowers.

To normalize blood pressure, from migraine, it is recommended to use an infusion of medicinal sweet clover. The course should be limited to 10-14 days.

To dilate blood vessels, improve blood flow, increase the resistance of capillaries and blood vessels, it is recommended to use decoctions of mushroom grass, periwinkle, nettle, burial ground.

Disease prognosis

Erythremia is considered a benign disease, but without adequate treatment, it can be fatal.

The prognosis of the disease depends on several factors:

• timely diagnosis of the disease - the earlier the disease is detected, the sooner treatment will begin;
• correctly prescribed treatment;
• the level of leukocytes, platelets and erythrocytes in the blood - the higher their level, the worse the prognosis;
• the body's response to treatment. Sometimes, despite treatment, the disease progresses;
• thrombotic complications;
• the rate of malignant transformation of the tumor.

In general, the prognosis for life with erythremia is positive. With timely diagnosis and treatment, patients can live more than 20 years from the moment the disease is detected.