hypererythremia. What is Erythremia. Diagnostic tests and procedures

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

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

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

Etiology

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

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

Kinds

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

acute, also called erythroleukemia;
chronic.

Depending on the progression of blood pathology:

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

Depending on the pathogenesis, true polycythemia is divided into:

primary;
secondary.

stages

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

Clinicians in the progression of erythremia note three stages:

initial;
erythremic;
anemic.

Initial

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

erythremic

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

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

anemic

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

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

Symptoms

Symptoms of erythremia directly depend on the stage of the disease. But it is also worth noting that some signs may appear at an early stage and not disappear until anemic.

Initial symptoms:

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

Symptoms of the erythremic stage:

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

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

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

Diagnostics

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

decrease in hematocrit and hemoglobin levels;

recovery normal level gland.

Medical therapy:

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

To reduce hemoglobin, they resort to erythrocytapheresis and bloodletting.

Symptomatic therapy:

antihistamine drugs;
antihypertensive agents;
cardiac glycosides;
gastroprotectors.

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

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

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

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

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

Causes of erythremia

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

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

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

Symptoms and signs of erythremia

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

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

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

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

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

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

Urine is unremarkable, although albuminuria is not uncommon due to general violation exchange or usually non-progressive dystrophic changes in the renal tissue.

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

Course, complications and clinical forms of erythremia

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

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

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

Treatment of erythremia

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

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

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

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

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 - quite rare disease. It is diagnosed in about 4 out of 100 million people a year. There is no dependence of the disease on the sex of a person, but it usually develops in patients after 50. 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 tend to heavy 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 human body) begin to be intensively produced so that they cannot fit in the bloodstream. When these red blood cells overflow the bloodstream, blood viscosity and thrombus formation increase. 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 cell clone is formed that has similar abilities for modification (it can become an erythrocyte, platelet or leukocyte), but is not controlled by the body's regulatory systems 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.

This is how 2 arise different types 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 leukocytes derived from cancer cell, is growing. 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 the 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 taking into account 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 limbs, there are 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 not so pronounced, so it can be taken as the norm. There may be a decrease mental capacity. Headache is not a specific sign of the disease, but is present at the initial stage due to poor 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 appear trophic ulcers. 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 seem 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 uric acid. Acute attacks may occur burning pains(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 peripheral vessels. Suffering nervous system, 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. Data deviations laboratory indicators 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 last stage are falling.

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. Except general analysis blood is carried 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.

To avoid iron deficiency, prescribe iron-containing preparations- "Maltofer", "Hemofer", "Sorbifer", "Totem", "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 products 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, migraine 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 capillary resistance 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.

What is Erythremia
What causes Erythremia
Symptoms of Erythremia
Diagnosis of Erythremia
Erythremia Treatment
Which doctors should you contact if you have Erythremia

What is Erythremia

erythremia - chronic leukemia with a lesion at the cell level - a precursor of myelopoiesis with an unrestricted growth of this cell characteristic of a tumor, which 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 causes 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

discovered violations of the chromosomal set are not predisposed to more malignant course diseases.

Although morphological, enzymatic and cytogenetic signs of lesions lymphatic system with erythremia is not available, functional state T-lymphocytes changed: a reduced response to known mitogens and an increase in their spontaneous activity were 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 enlarged, and the fatty marrow may appear red, hematopoietic.

The spleen is engorged, contains areas of infarcts of varying age, platelet aggregates, and often initial, moderate, or significant signs of myeloid metaplasia localized in the sinuses. The follicular structure is usually preserved.

In the liver, along with plethora, there are foci of fibrosis, the connection of the 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, shriveled 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. Increasing redness skin, weakness, heaviness in the head, enlarged spleen, arterial hypertension, 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 is more large arteries lower and upper limbs, 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 observed more often. 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 in the 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 can be acute leukemia, chronic myeloid leukemia, hypoplastic state of hematopoiesis and difficult to classify hematological changes.

Arterial hypertension, which 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 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 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, and 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.

Erythremia.
Secondary absolute erythrocytosis (due to increased formation of erythropoietins).
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.

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

Normal saturation of arterial blood with oxygen (more than 92%).
Enlargement of the spleen.
Leukocytosis more than 12 H 103 in 1 µl (in the absence of infections and intoxications).
Thrombocytosis more than 4 hours 105 in 1 µl (in the absence of bleeding).
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, a study is required ilium in order 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, with elevated levels of 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 500 ml every other day in the hospital and 2 days later when outpatient treatment. Instead of bloodletting, it is better to carry out erythrocytapheresis. The number of bleeds is determined by the achievement normal indicators red blood.

In elderly patients or those with accompanying illnesses of cardio-vascular 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. Elderly age 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 milurit (allopurinol) in daily dose from 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 wide application acetylsalicylic acid gastrointestinal bleeding, including prolonged and representing 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. Anemia caused by iron deficiency or folic acid appropriate replacement therapy is prescribed. Treatment of hemodilution anemia should be aimed at shrinking the spleen with radiotherapy, cytostatics and prednisolone. Anemia due to 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 steroid therapy, although its mechanism of action is not fully understood.

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 γ-therapy to the spleen area in a course dose of 5 Gy, sometimes a little more, if the number of platelets allows. Noticed positive action 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, but it is limited to the period of treatment and the immediate time after its cancellation.

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, which 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.

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

Although there are no morphological, enzymatic and cytogenetic signs of damage to the lymphatic system in erythremia, 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.

In addition to this classic variant, 3 more types of changes 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 enlarged, and the fatty marrow may appear red, hematopoietic.

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

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

The average life expectancy of platelets is often shortened, there is a negative relationship 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, enlargement of the 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.

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.

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.

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.

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.

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

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.

Erythremia.
Secondary absolute erythrocytosis (due to increased formation of erythropoietins).
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.

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

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

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.

Normal saturation of arterial blood with oxygen (more than 92%).
Enlargement of the spleen.
Leukocytosis more than 12 H 103 in 1 µl (in the absence of infections and intoxications).
Thrombocytosis more than 4 hours 105 in 1 µl (in the absence of bleeding).
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.

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, with elevated levels of 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. In smokers, the study of 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 the 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 of a high content of IgG receptors on the neutrophil membrane; 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).

Erythremia Treatment:

The task of treatment- normalization of the amount of hemoglobin to 140-150 g / l (85-90 units) 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.

Elderly patients or those with comorbidities of cardio-vascular 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 nicotinic acid preparations. 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, a serious condition of the 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.

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 both by unrecognized ulcerative lesions of the gastrointestinal tract, characteristic of erythremia and asymptomatic, and by 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 radiation therapy, cytostatics, and prednisone. 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.

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 γ-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.

Which doctors should you contact if you have Erythremia:

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

B12 deficiency anemia

Anemia due to impaired synthesis by utilization of porphyrins

Anemia due to a violation of the structure of globin chains

Anemia characterized by the carriage of pathologically unstable hemoglobins

Anemia Fanconi

Anemia associated with lead poisoning

aplastic anemia

Autoimmune hemolytic anemia

Autoimmune hemolytic anemia with incomplete heat agglutinins

Autoimmune hemolytic anemia with complete cold agglutinins

Autoimmune hemolytic anemia with warm hemolysins

Heavy chain diseases

Werlhof's disease

von Willebrand disease

Di Guglielmo's disease

Christmas disease

Marchiafava-Micheli disease

Rendu-Osler disease

Alpha heavy chain disease

gamma heavy chain disease

Shenlein-Henoch disease

Extramedullary lesions

Hairy cell leukemia

Hemoblastoses

Hemolytic uremic syndrome

Hemolytic anemia associated with vitamin E deficiency

Hemolytic anemia associated with deficiency of glucose-6-phosphate dehydrogenase (G-6-PDH)

Hemolytic disease of the fetus and newborn

Hemolytic anemia associated with mechanical damage to red blood cells

Hemorrhagic disease of the newborn

Histiocytosis malignant

Histological classification of Hodgkin's disease

DIC

Deficiency of K-vitamin-dependent factors

Factor I deficiency

Factor II deficiency

Factor V deficiency

Factor VII deficiency

Factor XI deficiency

Factor XII deficiency

Factor XIII deficiency

Iron-deficiency anemia

Patterns of tumor progression

Immune hemolytic anemias

Bedbug origin of hemoblastoses

Leukopenia and agranulocytosis

Lymphosarcomas

Lymphocytoma of the skin (Caesari disease)

Lymph node lymphocytoma

Lymphocytoma of the spleen

Radiation sickness

Marching hemoglobinuria

Mastocytosis (mast cell leukemia)

Megakaryoblastic leukemia

The mechanism of inhibition of normal hematopoiesis in hemoblastoses

Mechanical jaundice

Myeloid sarcoma (chloroma, granulocytic sarcoma)

multiple myeloma

Myelofibrosis

Violations of coagulation hemostasis

Hereditary a-fi-lipoproteinemia