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Wakez disease symptoms. Wakez disease: what is it, causes, symptoms, blood test, treatment So, the diagnosis is established ... What's next

Content

Hematologists know that this disease is difficult to treat and has dangerous complications. Polycythemia is characterized by changes in the composition of the blood that affect the health of the patient. How does the pathology develop, what are the symptoms? Find out diagnostic methods, methods of treatment, medicines, life forecasts for the patient.

What is polycythemia

Men are more susceptible to the disease than women, middle-aged people are more likely to get sick. Polycythemia is an autosomal recessive pathology in which, for various reasons, the number of red blood cells, or blood cells, increases in the blood. The disease has other names - erythrocytosis, polyhemorrhage, Wakez's disease, erythremia, its ICD-10 code is D45. The disease is characterized by:

splenomegaly - a significant increase in the size of the spleen;
increased blood viscosity;
significant production of leukocytes, platelets;
an increase in circulating blood volume (CBV).

Polycythemia belongs to the group of chronic leukemias and is considered a rare form of leukemia. True erythremia (polycythemia vera) is divided into types:

Primary - a malignant disease with a progressive form associated with hyperplasia of the cellular components of the bone marrow - myeloproliferation. Pathology affects the erythroblastic germ, which causes an increase in the number of red blood cells.
Secondary polycythemia is a compensatory reaction to hypoxia caused by smoking, alpine climbing, adrenal tumors, and pulmonary pathology.

Wakez's disease is dangerous with complications. Due to the high viscosity, blood circulation in the peripheral vessels is disturbed. Uric acid is excreted in large quantities. All this is fraught with:

bleeding;
thrombosis;
oxygen starvation of tissues;
hemorrhages;
hyperemia;
hemorrhage;
trophic ulcers;
renal colic;
ulcers in the digestive tract;
kidney stones;
splenomegaly;
gout;
myelofibrosis;
iron deficiency anemia;
myocardial infarction;
stroke
lethal outcome.

Types of disease

Wakez's disease, depending on developmental factors, is divided into types. Each has its own symptoms and treatment options. Physicians distinguish:

true polycythemia, which is caused by the appearance of a tumor substrate in the red bone marrow, leading to an increase in the production of red blood cells;
secondary erythremia - its cause is oxygen starvation, pathological processes occurring in the patient's body and causing a compensatory reaction.

Primary

The disease is characterized by tumor origin. Primary polycythemia is a myeloproliferative blood cancer that occurs when pluripotent stem cells in the bone marrow are damaged. When a disease occurs in the patient's body:

increases the activity of erythropoietin, which regulates the production of blood cells;
the number of erythrocytes, leukocytes, platelets increases;
there is a synthesis of mutated brain cells;
proliferation of infected tissues is formed;
there is a compensatory reaction to hypoxia - there is an additional increase in the number of erythrocytes.

With this type of pathology, it is difficult to influence mutated cells that have a high ability to divide. Thrombotic, hemorrhagic lesions appear. Wakez's disease has developmental features:

changes occur in the liver, spleen;
tissues are filled with viscous blood, prone to the formation of blood clots;
plethoric syndrome develops - cherry-red color of the skin;
arises severe itching;
rises arterial pressure(HELL);
hypoxia develops.

True polycythemia is dangerous for its malignant development, causing severe complications. For this form of pathology, the following stages are characteristic:

Initial - lasts about five years, is asymptomatic, the size of the spleen is not changed. BCC increased slightly.
Extended stage - duration up to 20 years. It is characterized by an increased content of erythrocytes, platelets, leukocytes. It has two substages - without changes in the spleen and with the presence of myeloid metaplasia.

The last stage of the disease - posterythremic (anemic) - is characterized by complications:

secondary myelofibrosis;
leukopenia;
thrombocytopenia;
myeloid transformation of the liver, spleen;
cholelithiasis, urolithiasis;
transient ischemic attacks;
anemia - the result of depletion of the bone marrow;
pulmonary embolism;
myocardial infarction;
nephrosclerosis;
leukemia in acute, chronic form;
hemorrhages in the brain.

Secondary polycythemia (relative)

This form of Wakez disease is provoked by external and internal factors. With the development of secondary polycythemia, viscous blood, which has increased volumes, fills the vessels, provoking the formation of blood clots. With oxygen starvation of tissues, a compensation process develops:

the kidneys begin to intensively produce the hormone erythropoietin;
active synthesis of erythrocytes in the bone marrow is launched.

Secondary polycythemia occurs in two forms. Each of them has features. The following varieties are distinguished:

stressful - caused by an unhealthy lifestyle, prolonged overvoltage, nervous breakdowns, unfavorable working conditions;
false, in which the total number of erythrocytes, leukocytes, platelets in the analyzes is within the normal range, an increase in ESR causes a decrease in plasma volume.

Causes

The provoking factors for the development of the disease depend on the form of the disease. Primary polycythemia occurs as a result of a neoplasm of the red bone marrow. The predetermining causes of true erythrocytosis are:

genetic failures in the body - a mutation of the tyrosine kinase enzyme, when the amino acid valine is replaced by phenylalanine;
hereditary predisposition;
cancerous tumors of the bone marrow;
oxygen deficiency - hypoxia.

The secondary form of erythrocytosis is caused by external causes. Concomitant diseases play an equally important role in development. The provoking factors are:

climatic conditions;
living in highlands;
congestive heart failure;
cancerous tumors of internal organs;
pulmonary hypertension;
the action of toxic substances;
overstrain of the body;
x-ray radiation;
insufficient supply of oxygen to the kidneys;
infections that cause intoxication of the body;
smoking;
bad ecology;
features of genetics - Europeans are more likely to get sick.

The secondary form of Wakez's disease is caused by congenital causes- autonomous production of erythropoietin, high affinity of hemoglobin for oxygen. There are also acquired factors for the development of the disease:

arterial hypoxemia;
kidney pathology - cystic lesions, tumors, hydronephrosis, stenosis of the renal arteries;
bronchial carcinoma;
adrenal tumors;
hemangioblastoma of the cerebellum;
hepatitis;
cirrhosis of the liver;
tuberculosis.

Symptoms of Wakez disease

A disease caused by an increase in the number of red blood cells and blood volume is distinguished characteristics. They have their own characteristics depending on the stage of Wakez's disease. Common symptoms of pathology are observed:

dizziness;
visual impairment;
Cooperman's symptom - a bluish hue of the mucous membranes and skin;
angina attacks;
redness of the lower and lower fingers upper limbs accompanied by pain, burning;
thrombosis of various localization;
severe itching of the skin, aggravated by contact with water.

As the pathology progresses, the patient develops pain syndromes of various localization. Violations are observed nervous system. For the disease are characteristic:

weakness;
fatigue;
temperature rise;
enlargement of the spleen;
noise in ears;
dyspnea;
feeling of loss of consciousness;
plethoric syndrome - burgundy-red color of the skin;
headaches;
vomit;
increase in blood pressure;
pain in the hands from touch;
chilliness of the limbs;
eye redness;
insomnia;
pain in the hypochondrium, bones;
pulmonary embolism.

initial stage

The disease is difficult to diagnose at the very beginning of development. The symptoms are mild, similar to a cold or a condition of the elderly, corresponding to advanced age. Pathology is detected by chance during tests. The symptoms of the initial stage of erythrocytosis are:

dizziness;
decrease in visual acuity;
headache attacks;
insomnia;
noise in ears;
sore fingers from touch;
cold extremities;
ischemic pain;
redness of mucous surfaces, skin.

Expanded (erythremic)

The development of the disease is characterized by the appearance pronounced signs high blood viscosity. Pancytosis is noted - an increase in the number of components in the analyzes - erythrocytes, leukocytes, platelets. The advanced stage is characterized by the presence of:

reddening of the skin to purple shades;
telangiectasia - pinpoint hemorrhages;
acute attacks of pain;
itching, aggravated by contact with water.

At this stage of the disease, signs of iron deficiency are observed - stratification of the nails, dry skin. characteristic symptom- a strong increase in the size of the liver, spleen. Patients have:

indigestion;
breathing disorder;
arterial hypertension;
pain in the joints;
hemorrhagic syndrome;
microthrombosis;
stomach ulcers, duodenum;
bleeding;
cardialgia - pain in the left chest;
migraine.

With an advanced stage of erythrocytosis, patients complain of a lack of appetite. Investigations reveal stones in gallbladder. The disease is different

increased bleeding from small cuts;
violation of the rhythm, conduction of the heart;
puffiness;
signs of gout;
pain in the heart;
microcytoses;
symptoms of urolithiasis;
changes in taste, smell;
bruising on the skin;
trophic ulcers;
renal colic.

anemic stage

At this stage of development, the disease passes into the terminal stage. The body does not have enough hemoglobin to function properly. The patient has:

a significant increase in the liver;
progression of splenomegaly;
compaction of the tissues of the spleen;
with hardware research - cicatricial changes in the bone marrow;
vascular thrombosis of deep veins, coronary, cerebral arteries.

At the anemic stage, the development of leukemia is a danger to the patient's life. This stage of Wakez's disease is characterized by the occurrence of aplastic iron deficiency anemia, the cause of which is the displacement of hematopoietic cells from the bone marrow. connective tissue. In this case, symptoms are observed:

general weakness;
fainting;
feeling of lack of air.

At this stage, if left untreated, the patient quickly dies. Thrombotic, hemorrhagic complications lead to it:

ischemic form of stroke;
thromboembolism of the pulmonary arteries;
myocardial infarction;
spontaneous bleeding - gastrointestinal, veins of the esophagus;
cardiosclerosis;
arterial hypertension;
heart failure.

Symptoms of the disease in newborns

If the fetus has suffered hypoxia during fetal development, its body, in response, begins to increase the production of red blood cells. A provoking factor in the appearance of erythrocytosis in infants is congenital heart disease, pulmonary pathologies. The disease leads to the following consequences:

the formation of bone marrow sclerosis;
impaired production of leukocytes responsible for immune system newborn;
development of infections leading to death.

At the initial stage, the disease is detected by the results of tests - the level of hemoglobin, hematocrit, red blood cells. With the progression of the pathology, pronounced symptoms are observed already in the second week after birth:

the baby cries from touch;
the skin turns red;
the size of the liver, spleen increases;
thrombosis appears;
body weight decreases;
analysis reveals increased amount erythrocytes, leukocytes, platelets.

Diagnosis of polycythemia

The patient's communication with the hematologist begins with a conversation, an external examination, and anamnesis. The doctor finds out heredity, features of the course of the disease, the presence of pain, frequent bleeding, signs of thrombosis. During the reception, the patient is diagnosed with polycythemic syndrome:

purple-red blush;
intense coloring of the mucous membranes of the mouth, nose;
cyanotic (cyanotic) color of the palate;
change in the shape of the fingers;
red eyes;
palpation is determined by an increase in the size of the spleen, liver.

The next stage of diagnosis is laboratory research. Indicators that indicate the development of the disease:

an increase in the total mass of erythrocytes in the blood;
increase in the number of platelets, leukocytes;
a significant level of alkaline phosphatase;
a large amount of vitamin B 12 in the blood serum;
increased erythropoietin in the secondary form of polycythemia;
decrease in situation (blood oxygen saturation) - less than 92%;
decrease in ESR;
increase in hemoglobin to 240 g / l.

For differential diagnosis pathology, special types of research and analysis are used. Urologist, cardiologist, gastroenterologist are consulted. The doctor prescribes:

biochemical analysis blood - determines the level of uric acid, alkaline phosphatase;
radiological examination - reveals an increase in circulating red blood cells;
sternal puncture - sampling for cytological analysis of the bone marrow from the sternum;
trepanobiopsy - histology of tissues from the ilium, revealing three-growth hyperplasia;
molecular genetic analysis.

Laboratory research

The disease of polycythemia is confirmed by hematological changes in blood parameters. There are parameters that characterize the development of pathology. Laboratory data indicating the presence of polycythemia:

Indicator	Units	Meaning
Hemoglobin
Mass of circulating red blood cells
Erythrocytosis	cells/ liter
Leukocytosis		over 12x109
thrombocytosis		over 400x109
Hematocrit

Serum vitamin B level 12
Alkaline phosphatase		over 100
Color indicator

Hardware diagnostics

After conducting laboratory tests, hematologists prescribe additional tests. To assess the risk of developing metabolic, thrombohemorrhagic disorders, hardware diagnostics are used. The patient undergoes research depending on the characteristics of the course of the disease. A patient with polycythemia is given:

Ultrasound of the spleen, kidneys;
heart examination - echocardiography.

Methods of hardware diagnostics help to assess the condition of the vessels, to identify the presence of bleeding, ulcers. Appointed:

fibrogastroduodenoscopy (FGDS) - an instrumental study of the mucous membranes of the stomach, duodenum;
ultrasound dopplerography (USDG) of the vessels of the neck, head, veins of the extremities;
computed tomography of internal organs.

Treatment of polycythemia

Before proceeding with therapeutic measures, it is necessary to find out the type of disease and its causes - the treatment regimen depends on this. The challenge for haematologists is to:

in primary polycythemia, prevent tumor activity by influencing the neoplasm in the bone marrow;
in the secondary form - to identify the disease that provoked the pathology and eliminate it.

Treatment of polycythemia includes drawing up a rehabilitation and prevention plan for a particular patient. Therapy includes:

bloodletting, which reduces the number of red blood cells to normal - 500 ml of blood is taken from the patient every two days;
maintaining physical activity;
erythocytophoresis - blood sampling from a vein, followed by filtration and return to the patient;
dieting;
transfusion of blood and its components;
chemotherapy to prevent leukemia.

In difficult situations that threaten the life of the patient, a bone marrow transplant is performed, a splenectomy is the removal of the spleen. In the treatment of polycythemia, much attention is paid to the use of medications. The treatment regimen includes the use of:

corticosteroid hormones - with a severe course of the disease;
cytostatic agents - Hydroxyurea, Imiphos, which reduce the growth of malignant cells;
antiplatelet agents that thin the blood - Dipyridamole, Aspirin;
Interferon, which increases defenses, enhances the effectiveness of cytostatics.

Symptomatic treatment involves the use of drugs that reduce blood viscosity, prevent thrombosis, and the development of bleeding. Hematologists prescribe:

to exclude vascular thrombosis - Heparin;
with severe bleeding - Aminocaproic acid;
in the case of erythromelalgia - pain in the fingertips - non-steroidal anti-inflammatory drugs - Voltaren, Indomethacin;
with skin itching - antihistamines- Suprastin, Loratadin;
with an infectious genesis of the disease - antibiotics;
for hypoxic causes - oxygen therapy.

Bloodletting or erythrocytopheresis

An effective way to treat polycythemia is phlebotomy. When bloodletting is performed, the volume of circulating blood decreases, the number of red blood cells (hematocrit) decreases, and skin itching is eliminated. Features of the process:

before phlebotomy, the patient is administered Heparin or Reopoliglyukin to improve microcirculation, blood flow;
excess is removed with leeches or an incision is made, a puncture of a vein;
up to 500 ml of blood is removed at a time;
the procedure is carried out with an interval of 2 to 4 days;
hemoglobin is reduced to 150 g/l;
the hematocrit is adjusted to 45%.

Another method of treating polycythemia, erythrocytopheresis, is effective. With extracorporeal hemocorrection, excess red blood cells are removed from the patient's blood. This improves the processes of hematopoiesis, increases the consumption of iron by the bone marrow. Scheme for performing cytopheresis:

Create vicious circle- in a patient, the veins of both hands are connected through a special apparatus.
Blood is taken from one.P
It is passed through an apparatus with a centrifuge, a separator, filters, where some of the red blood cells are eliminated.
The purified plasma is returned to the patient - injected into a vein in the other arm.

Myelosuppressive therapy with cytostatics

In severe cases of polycythemia, when bloodletting does not give positive results, doctors prescribe drugs that suppress the formation and reproduction of brain cells. Treatment with cytostatics requires ongoing blood tests to monitor the effectiveness of therapy. Indications are factors associated with polycythemic syndrome:

visceral, vascular complications;
skin itching;
splenomegaly;
thrombocytosis;
leukocytosis.

Hematologists prescribe drugs based on the results of tests, the clinical picture of the disease. Contraindications for cytostatic therapy are childhood. Drugs used to treat polycythemia:

Myelobramol;
Imiphos;
Cyclophosphamide;
Alkeran;
Mielosan;
Hydroxyurea;
Cyclophosphamide;
Mitobronitol;
Busulfan.

Preparations for the normalization of the aggregate state of the blood

The objectives of treatment for polycythemia: normalization of hematopoiesis, which includes ensuring the liquid state of the blood, its coagulation during bleeding, restoration of the walls of blood vessels. Doctors have a serious choice of drugs so as not to harm the patient. Prescribe medications that help stop bleeding - hemostatics:

coagulants - Thrombin, Vikasol;
fibrinolysis inhibitors - Kontrykal, Amben;
vascular aggregation stimulants - calcium chloride;
drugs that reduce permeability - Rutin, Adroxon.

Of great importance in the treatment of polycythemia to restore the aggregate state of the blood is the use of antithrombotic agents:

anticoagulants - Heparin, Hirudin, Phenylin;
fibrolytics - Streptoliasis, Fibrinolysin;
antiplatelet agents: platelet - Aspirin (Acetylsalicylic acid), Dipyridamole, Indobrufen; erythrocyte - Reogluman, Reopoliglyukin, Pentoxifylline.

recovery prognosis

What awaits a patient diagnosed with polycythemia? Forecasts depend on the type of disease, timely diagnosis and treatment, causes, and complications. Wakez's disease in its primary form has an unfavorable development scenario. Life expectancy is up to two years, which is associated with the complexity of therapy, high risks formation of strokes, heart attacks, thromboembolic consequences. Survival can be increased by using the following treatments:

local irradiation of the spleen with radioactive phosphorus;
lifelong bloodletting procedures;
chemotherapy.

A more favorable prognosis for the secondary form of polycythemia, although the disease may result in nephrosclerosis, myelofibrosis, erythrocyanosis. Although a complete cure is impossible, the patient's life is extended for a significant period - over fifteen years - provided:

constant monitoring by a hematologist;
cytostatic treatment;
regular hemocorrection;
undergoing chemotherapy;
elimination of factors provoking the development of the disease;
treatment of pathologies that caused the disease.

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With a disease, there is a danger of hypoxia, when the tissues of the body receive less oxygen. Even with excellent immunity, the patient needs treatment. The body itself can not cope with the disease:

there are problems with the liver and spleen;
blood pressure rises;
the proper functioning of the kidneys is disrupted.

Note!

The elderly are at risk. Most often, according to statistics, these are men.

If younger people or children get sick, they endure it much harder. The causes of the disease lie in the genetic predisposition of people. Wakez-Osler disease is named after Frenchman Louis Henry Vakez and Canadian William Osler, doctors who first described the disease in the late 19th and early 20th centuries.

Stages and symptoms of Wakez disease

This disease develops secretly. Many patients do not even know about it for a long time. At the beginning of the disease, there are no specific symptoms.

At the middle stage:

There are complaints of constant headaches.
Dizzy, hard to concentrate.
There is severe itching of the epidermis.
The skin turns red, the veins are translucent.
Often itching is aggravated by contact with water.
In addition to redness of the skin, there is also a darkening of the mucous membrane.
The veins in the neck swell and become visible.

Platelets are the main cause of clogging of capillaries, so patients experience pain in the limbs and joints. Bleeding gums are often observed. Patients lose weight, catch a cold. They have a decrease in visual acuity, hearing, frequent mood swings.

The next stage is accompanied by dehydration, oxygen starvation, and lung disease. Heart failure appears and kidney function worsens. Due to the lack of oxygen, the production of red blood cells increases.

The terminal stage is the beginning of the malignant stage of development. In addition to the syndrome of erythrocytosis, the patient feels pain throughout the body. Leukemia starts. Bleeding opens, inflammation and infection worsen. The spleen suffers, liver problems appear.

The mechanism of development and causes of the disease

At increased production erythrocytes significantly increases hemoglobin. A tumor forms in the bone marrow and the production of young red blood cells increases. Pathology leads to thickening of the blood. This secondary erythremia differs from the primary.

The causes of polycythemia are not well understood. The disease occurs due to mutations and increased creation of red blood cells (erythropoiesis).

On a note!

Since stem cell mutations develop in the bone marrow, doctors refer to this disease as a type of genetic pathology.

This is also evidenced by medical statistics. Varieties of chronic leukemia are often observed among relatives. The cause of Wakez may also be blood clotting disorders.

Symptoms and Diagnosis

Diagnosis at the beginning of the disease is difficult to make. Many of the symptoms of Wakez are characteristic of other ailments: joint disease appears as in gout, hot water causes itching. The veins become visible and swell. The skin becomes dark red. Appear:

dizziness;
weakness;
headache.

Patients have problems with vision, hearing, it is difficult for them to concentrate on something. The blood pressure rises. Perhaps the appearance of a stomach ulcer. Thrombi often form. The brain does not receive the necessary oxygen and the risk of a stroke increases. When anemia occurs, the skin turns pale, dizzy.

Attention!

To detect the disease, a biochemical blood test is used. The number of red blood cells in patients may exceed the norm by 2 times. This may indicate the onset of the disease.

Suspicion of polycythemia causes an increase in uric acid in the body. However, to be sure of the correctness of the diagnosis, a puncture of the brain is performed. The operation is performed without anesthesia. The puncture allows you to find out the state of fibrosis and cancer cells. To determine the degree of damage to internal organs, ultrasound examinations are carried out. The diagnosis is made if there is a persistent weight loss and the color of the skin and mucous membrane changes.

In a radiological examination, the number of red blood cells is counted. Trepanobiopsy examines the condition of the ilium. This method most accurately allows you to make a diagnosis. The bone marrow is examined by sternal puncture. Dopplerography is used to study the velocity of blood flow in the vessels.

Prevention and treatment

There are no effective measures to prevent this disease. This is about secondary prevention. This is a dynamic observation through examinations, treatment. Therefore, prevention is aimed at secondary types of the disease. First of all, you need:

stop smoking;
maintain body weight;
drink more water.

Everything needs to be healed acute diseases to control inflammation. Follow the recommendations of doctors, follow the prescribed diet. Usually this is "Table #5 and #6".

When choosing a treatment strategy, the age of the patient is taken into account. It is especially important to prevent the development of thrombosis. Most effective method- bleeding. Up to 500 ml of blood is pumped out at a time. Often this method is combined with chemotherapy. Cytoreductive therapy has a good effect. Used drugs such as:

Chlorambucil;
Mielosan.

Treatment of patients under 50 years of age, without complications, consists only of bloodletting. Well proven:

Alpha interferon;
anticancer drugs.

Of the medicines, Allopurinol is most often prescribed. At the same time, the work of the kidneys improves, the pain in the extremities disappears. Anticoagulants are prescribed to improve blood circulation. With skin itching, drugs that suppress the action of histamine are indicated. A hematologist draws up a separate treatment regimen. Aspirin eliminates circulatory disorders. The appointment of such antiplatelet agents is recommended for all patients.

The main recommendation is proper nutrition, dieting. In addition, physical overwork should not be allowed. Avoid foods that increase blood flow. Under the ban are cayenne pepper, pomegranates, onions. Useful vegetable, dairy products. In the second stage of the disease, it is necessary to exclude from food the consumption of:

fishes;
meat.

Note!

Even supporters traditional medicine agree that herbs will not cure the disease. Folk recipes are good for prolonging remission, maintaining a stable condition of the patient.

Lots of folk recipes have a good effect on the hematopoietic component. They definitely need to be used. Via folk methods can dull pain or relieve spasm. Full recovery with the help of herbal medicine is impossible. Even with the improvement of the symptoms of the disease, the pathology will not go anywhere, an exacerbation can occur at any time.

Traditional medicine can only dull the pain for a while. It can also relieve spasm, exacerbation. Recipes for herbal treatment have proven themselves well. For example, there are many ways to avoid emptying the blood. Grass Sweet clover is brewed in water, filtered through a sieve, consumed inside half a cup. Conduct a monthly course, then take a break for 2 months.

A wonderful effect gives the use of cranberries. For a glass of boiling water, you need 2 tablespoons of dry or fresh berries. Within half an hour, the infusion is brewed and ready for use. The decoction can be sweetened, consumed orally 30 minutes before meals.

Forecast

Polycythemia is a rare disease, the number of newly diagnosed patients per year is about 1 per 100,000 population. Statistics show that with a diagnosis of Vaquez, people live 10-20 years. The disease often affects the elderly. If the diagnosis was made at the age of 70-80, then a person can live on without much change in lifestyle. But general rules does not exist here.

The medical prognosis for polycythemia is favorable, since it is a benign disease. There is a chance of a complete cure. But no one is immune from recurrence of the disease. Perhaps the appearance of cirrhosis of the liver. Often occurs:

ischemic disease;
thromboembolism.

Doctors are not inclined to compare polycythemia with oncology. This disease is successfully treated. Oncology is the growth of the epithelium, and Wakeza-Osler is the defeat of the hematopoietic tissue. The disease does not develop quickly. A person during his life must take tests, experts see deviations in the state of the blood.

Timely detection of polycythemia can cure the patient on early stage. Diagnostics has improved significantly in recent years. Scientists have created a new class of drugs.

Polycythemia is a chronic disease in which there is an increase in the number of red cells or red blood cells in the blood. The disease affects people of middle and older age - men are affected several times more often than women. More than half of people have an increase in the number of platelets and leukocytes.

The occurrence of a disease can be due to several reasons for which its species are divided. Primary or true polycythemia is caused mainly by genetic abnormalities or bone marrow tumors, while secondary polycythemia is promoted by external or internal factors. Without proper treatment, it leads to severe complications, the prognosis of which is not always comforting. So, the primary form, if therapy is not started in a timely manner, can be fatal over several years of progression, and the outcome of the secondary depends on the cause of its occurrence.

The main symptoms of the disease are attacks of severe dizziness and tinnitus, it seems to a person that he is losing consciousness. Bloodletting and chemotherapy are used for treatment.

A distinctive feature of this disorder is that it cannot spontaneously disappear and it is also impossible to completely recover from it. A person will need to regularly take blood tests and be under the supervision of doctors until the end of his life.

Etiology

The causes of the disease depend on its form and can be caused by various factors. True polycythemia is formed when:

hereditary predisposition to violations of production;
genetic failures;
malignant neoplasms in the bone marrow;
exposure to hypoxia (oxygen deficiency) on red blood cells.

Secondary polycythemia is caused by:

chronic heart failure;
insufficient supply of blood and oxygen to the kidneys;
climatic conditions. The most affected are people living in high mountain areas;
oncological tumors of internal organs;
various infectious diseases that cause intoxication of the body;
harmful working conditions, for example, in a mine or at a height;
living in polluted cities or near factories;
long-term abuse of nicotine;
nation. According to statistics, polycythemia manifests itself in people of Jewish origin, this is due to genetics.

The disease itself is rare, but polycythemia is even rarer in newborns. The main way the disease is transmitted is through the mother's placenta. The child's place does not provide a sufficient supply of oxygen to the fetus (inadequate blood circulation).

Varieties

As mentioned above, the disease is divided into several types, which directly depend on the causes of occurrence:

primary or true polycythemia - caused by blood pathologies;
secondary polycythemia, which can be called relative - due to external and internal pathogens.

True polycythemia, in turn, can occur in several stages:

initial, which is characterized by a slight manifestation of symptoms or their complete absence. Can take up to five years
deployed. It is divided into two forms - without a malignant effect on the spleen and with its presence. The stage lasts one or two decades;
severe - observed, education cancerous tumors on the internal organs, including the liver and spleen, malignant lesions of the blood.

Relative polycythemia is:

stressful - based on the name, it becomes clear that it occurs when the body is affected by prolonged overvoltage, adverse working conditions and an unhealthy lifestyle;
false - in which the level of erythrocytes, and in the blood is within the normal range.

The prognosis of polycythemia vera is considered unfavorable, life expectancy with this disease does not exceed two years, but the chances of long life increase when used in the treatment of bloodletting. In this case, a person can live fifteen or more years. The prognosis of secondary polycythemia depends entirely on the course of the disease that triggered the process of increasing the number of red cells in the blood.

Symptoms

At the initial stage, polycythemia occurs with little or no symptoms. It is usually discovered during a random examination or during a preventive blood test. The first symptoms may be mistaken for a common cold or indicate a normal condition in the elderly. These include:

decreased visual acuity;
severe dizziness and headache attacks;
noise in ears;
sleep disturbance;
cold fingertips.

In the advanced stage, the following symptoms may be observed:

muscle and bone pain;
an increase in the size of the spleen, the volume of the liver changes a little less often;
bleeding gums;
continuous bleeding for quite a long time after tooth extraction;
the appearance of bruises on the skin, the nature of which a person cannot explain.

In addition, specific symptoms this disease are:

severe itching of the skin, which is characterized by an increase in intensity after taking a bath or shower;
painful burning sensations of the tips of the fingers and toes;
manifestation of veins that were not previously visible;
the skin of the neck, hands and face takes on a bright red color;
lips and tongue become bluish;
the whites of the eyes are filled with blood;
general weakness of the patient's body.

In newborns, especially twins, the symptoms of polycythemia begin to be expressed a week after birth. These include:

redness of the skin of the crumbs. The child begins to cry and scream during touching;
significant weight loss;
a large number of erythrocytes, leukocytes and platelets are found in the blood;
enlargement of the liver and spleen.

These signs can lead to the death of the baby.

Complications

The consequences of ineffective or untimely treatment can be:

excretion of large amounts of uric acid. Urine becomes concentrated and acquires an unpleasant odor;
education ;
chronic;
occurrence and ;
circulatory disorders leading to trophic ulcers on the skin;
hemorrhages in various places of localization, for example, nose, gums, gastrointestinal tract, etc.

And they are considered the most common causes of death of patients with such an ailment.

Diagnostics

Polycythemia is very often discovered by chance during a blood test for completely different reasons. When diagnosing, the doctor must:

carefully read the medical history of the patient and his immediate family;
conduct a complete examination of the patient;
find out the cause of the disease.

The patient, in turn, must undergo the following examinations:

Treatment of the primary disease is a rather laborious process, which includes the impact on tumors and the prevention of their activity. In therapy medicines the age of the patient plays an important role, because those substances that will help people under fifty will be strictly prohibited for the treatment of patients over seventy.

With a high content of erythrocytes in the blood in the best possible way treatment is bloodletting - during one procedure, the blood volume is reduced by about 500 milliliters. More modern method The treatment for polycythemia is considered to be cytopheresis. The procedure is to filter the blood. To do this, catheters are inserted into the veins of both arms of the patient, through one blood enters the apparatus, and after filtration, the purified blood returns to the other vein. This procedure must be carried out every other day.

For secondary polycythemia, treatment will depend on the underlying disease and the severity of its symptoms.

Prevention

Most causes of polycythemia cannot be prevented, but despite this, there are several preventive measures:

completely stop smoking;
change place of work or residence;
timely treat diseases that can cause this disorder;
regularly undergo preventive examinations in the clinic and take a blood test.

The disease "true polycythemia" is a tumor process that affects the blood system and is benign. Synonyms for this name erythremia, primary polycythemia, Wakez disease. The pathological process is associated with hyperplasia of the cellular elements of the bone marrow (myeloproliferation). As a result, the amount in the blood increases significantly, and the level of neutrophils also increases. Due to the increase in the number of red blood cells, the mass of circulating blood also becomes larger, it becomes more viscous. As a result, blood circulation slows down, blood clots form, which leads to impaired blood supply. However, for many years, erythremia can occur without severe symptoms.

Most often, erythremia affects the elderly, but it also occurs in young people and children. In young people, the disease is more severe. Men get sick more often than women. There is a genetic predisposition to the development of this disease. The disease code "erythremia" according to MBK-10 is D45. According to medical statistics, the prevalence of Wakez disease is 29 cases per 100,000 population.

Pathogenesis

Polycythemia vera is a clonal neoplastic disease, the basis of which is the process of transformation of the hematopoietic stem cell. Due to a defect in such a cell, a somatic mutation occurs in the Janus kinase receptor gene. cytokines. This leads to the proliferation of myeloid hematopoietic sprouts, mainly erythrocyte, which increases the risk of thrombosis and thromboembolism.

Prolonged proliferation of hematopoietic cells provokes the development of fibrosis and replacement of the bone marrow with collagen fibers, resulting in the development of secondary postpolycythemic myelofibrosis. In some cases, the progression of the disease continues, and it enters the phase of blast transformation.

A characteristic symptom of the disease is the presence of clusters of polymorphic megakaryocytes, both small and giant.

In the course of the development of the disease, an increase in the mass of circulating erythrocytes is noted, increases, blood viscosity becomes pronounced, and the level increases significantly. Due to these factors and thrombocytosis, microcirculation is disturbed and thromboembolic complications occur. joins the process myeloid metaplasia spleen.

In a fairly significant proportion of patients at the stage of myelofibrosis, chromosomal abnormalities are determined.

Classification

There are two forms of polycythemia - true And relative.

True erythremia, in turn, can be primary And secondary.

Primary erythremia is an independent myeloproliferative disease in which the myeloid germ of hematopoiesis is affected.
Secondary polycythemia- appears when the activity of erythropoietin increases. Secondary erythrocytosis (also called "symptomatic erythrocytosis") is a compensatory reaction to the general. Symptomatic erythrocytosis may develop in patients with chronic form lung pathology, tumors of the adrenal glands, heart defects, etc. Physiological erythrocytosis is sometimes noted during ascent to a height.

The relative form of the disease is noted if the level of erythrocytes is normal, but plasma volume decreases. This state is also called false or stressful. polycythemia.

Causes

Until now, the causes of erythrocytosis have not been precisely determined. Currently, scientists have confirmed that the causes of the development of the disease are associated with a hereditary factor.

In addition, the following external factors can provoke the development of this disease:

Exposure to the body of toxic substances - varnishes, paints, chemical insecticides.
Prolonged use of certain drugs - gold preparations.
Postponed, some viral diseases.
Influence of radioactive radiation.

As the disease progresses, the symptoms of Wakez disease change. The symptoms of polycythemia vera depend on the stage of the disease. Doctors distinguish four stages of the disease, reflecting the pathological changes that occur in the spleen and bone marrow.

The first stage is initial. It can last five or more years. During this period, moderate erythrocytosis and moderate plethora are noted, the spleen is not palpable at this stage. Panmyelosis is noted in the bone marrow. There is a low probability of vascular and thrombotic complications. Outwardly manifested acrocyanosis, plethora, erythromelalgia(paresthesia and burning in the tips of the fingers), itching of the skin after washing. When the total volume of circulating blood increases, arterial hypertension. At the same time, treatment with antihypertensive drugs in people who have not previously suffered from hypertension is ineffective. Gradually develop manifestations of cerebral, coronary heart disease. Patients complain of headaches, pain in the fingers when moving.
The second stage is expanded, erythremic. It can be observed for 10-15 years. If this stage proceeds without myeloid metaplasia of the spleen, then the patient's general state, there is a pronounced plethora, panmyelosis. There is a high probability of thrombotic complications - fingertips,. The patient may be disturbed by pain in the bones, arms and legs. If the patient has myeloid metaplasia of the spleen, hepatosplenomegaly and panmyelosis are observed. Moderately pronounced plethora, increased bleeding. Thrombotic complications are likely. The skin becomes purple-blue, burning pains in the fingertips, earlobes disturb. There may be a violation of sensitivity. There may be pain in the hypochondrium due to an increase in the liver and spleen, bleeding gums, joint pain, and ulcerative disorders of the gastrointestinal tract are likely.
The third stage is anemic. This is the terminal period in which an anemic syndrome develops, expressed. The liver and spleen are enlarged. Increases collagen myelofibrosis in the bone marrow. are growing cachexia And splenomegaly, manifestations of plethora are noted, as well as complications provoked by thrombosis. Hemorrhages occur in different organs, the severity is aggravated common symptoms.

Thus, true erythremia can provoke the following symptoms:

Discoloration of the skin and vasodilation - dilated veins are especially noticeable in the neck, sometimes in other places. The skin with polycythemia is red-cherry. Color changes are especially pronounced in its open areas. The lips and tongue acquire a bluish tint, the conjunctiva of the eyes is hyperemic.
Itching of the skin - almost half of the patients have skin itching, which increases after exposure to warm water. This effect is associated with release processes, histamine, .
The presence of common symptoms - patients complain of headaches, severe fatigue, tinnitus, the appearance of "flies" before the eyes, etc. Blood pressure rises, and sometimes heart failure develops.
Splenomegaly(enlargement of the spleen) - manifests itself in varying degrees. Hepatomegaly (enlargement of the liver) is also likely.
erythromelalgia- strong burning pain in the tips of the fingers of the extremities. The manifestations are short-lived. At the same time, the skin turns red and cyanotic spots of purple color appear. The pain is associated with an increase in the level of platelets and the development of microthrombi in the capillaries.
Ulcers in the stomach and duodenum- a symptom is observed relatively rarely (in about 15% of cases), it is associated with capillary thrombosis and trophic disorders of the gastrointestinal mucosa, leading to a decrease in its resistance to exposure Helicobacter pylori.
Bleeding- in addition to increased blood clotting and the formation of blood clots, bleeding occurs from the gums and dilated vessels of the esophagus.
Thrombi in blood vessels due to increased blood viscosity pathological changes blood clots form in the walls of blood vessels and thrombocytosis, blood circulation is disturbed.
Pain in the legs and joints- to pain in the limbs lead obliterating endarteritis And erythromelalgia; to pain in the joints - an increase in uric acid.
Pain in the bones- develop against the backdrop bone marrow hyperplasia.

Analyzes and diagnostics

To establish the diagnosis, a laboratory blood test and additional examinations (ultrasound, CT) are performed. To make a correct diagnosis, doctors evaluate blood test results. Erythremia is determined if certain indicators are available, basic and additional. Erythremia is suspected if there are abnormalities in the general blood test, in particular, hemoglobin is elevated (more than 18.5 g / dl in men, more than 16.5 g / dl in women). Attention is drawn to a number of other indicators ( erythrocytosis, thrombocytosis, sometimes - pancytosis).

The main criteria for establishing such a diagnosis are:

An increase in the number of red blood cells (in men - above 36 ml / kg, in women - above 32 ml / kg).
Normal oxygen saturation of arterial blood (more than 92%).
Splenomegaly.

Additional criteria are:

Leukocytosis(above 12 x 109/l without signs of infection).
thrombocytosis(above 400 x 109/l).
Elevated level (above 900 pg / ml).
Activity alkaline phosphatase.

Other laboratory parameters are also analyzed. To confirm the diagnosis, the histological picture of the brain is evaluated.

In addition, the following factors are important in establishing a diagnosis:

The characteristic appearance of the patient is the presence of a specific color of the skin and mucous membranes.
Enlarged spleen, liver.
Tendency to form blood clots.
The presence of general symptoms characteristic of polycythemia vera.

Differential diagnosis is carried out with secondary erythrocytosis.

Treatment of polycythemia

Treatment is carried out in such a way as to reduce the viscosity of the patient's blood and minimize the risk of serious complications - the formation of blood clots and bleeding.

The doctors

Medications

In order to facilitate the process of bloodletting, which is prescribed for treatment, as well as for the prevention of thrombotic complications, courses of antiplatelet treatment are carried out. For this purpose drugs are used Ticlopidin, . The use of aspirin can reduce the severity of microvascular disorders.

For the prevention of blood clotting is prescribed.

Drugs that lower the number of platelets are also used. For this purpose, appoint Interferon-alpha, Anagrelide. A non-specific JAK inhibitor is also used Ruxolitinib.

Patients at an older age (70 years and older) are prescribed myelosuppressive drugs. For chemotherapy is prescribed Chlorambucil, Hydroxyurea, however, there is now an opinion that their long-term use can provoke the development of leukemia. The drug is also used Busulfan.

Conducted if necessary symptomatic treatment. For example, to reduce the severity of itching of the skin, antihistamines (,) and local preparations are used. In autoimmune hemolytic anemia, corticosteroid hormones are prescribed. To lower uric acid levels, anti-gout drugs are prescribed (, Febuxostat, ). High blood pressure removed with antihypertensive drugs. For ulcerative lesions of the gastrointestinal tract, they are used, etc.

Procedures and operations

During the treatment of polycythemia on her initial stages bloodletting (phlebotomy) is used, with the help of which it is possible to significantly reduce the manifestations of the plethoric syndrome. This procedure is carried out in the same way as the collection of donor blood. Phlebotomy allows you to reduce the volume of circulating blood, normalize. Once every 2-4 days, 300 to 500 ml of blood is removed. The procedure is carried out until the disappearance of the plethoric syndrome.

In modern clinics, bloodletting is replaced erythrocytepheresis. This procedure involves taking a certain number of red blood cells from the blood.

Currently does not exist effective therapy for the treatment of anemic, third stage of the disease. In this case, palliative care is used. Transfusions of blood components are also carried out to correct anemic and hemorrhagic syndromes.

Treatment with folk remedies

Polycythemia vera, the symptoms and treatment of which are described above, is a very serious disease that requires specialist treatment and constant monitoring. Therefore, treatment folk remedies better not to practice. Nevertheless, this or that forum of patients with erythremia often contains recommendations on the use of drugs that affect the composition of the blood. It is important to understand that any of the methods can only be used as additional remedy to improve the condition, in no case replacing the main treatment. For those who are interested in how polycythemia vera is treated with folk remedies, the forum suggests using the following methods:

Infusion of sweet clover. 10 g of dry grass should be poured with a glass of boiling water. After a few hours, strain, drink half a glass twice a day for a month.
Cranberry. Pour 30 g of dry or fresh berries with 1 glass of water and boil. Keep warm for half an hour. Drink with honey instead of tea.
Garlic. Twist 3 heads of garlic in a meat grinder, pour the mass into a jar and pour water. Insist 15 days in a dark place. Strain the liquid, add the juice of 2 lemons and 50 g of honey. Mix, consume 1 tbsp. l. before bedtime.
chestnuts. Peel of chestnuts should be crushed and pour about 50 g of raw materials with 500 ml of vodka. Infuse for half a month, then filter and consume 1 tbsp. l. diluted in a small amount of water three times a day. The course lasts three weeks.
Fresh juices. Every day, consume 100 g of fresh orange juice. It is also recommended to drink a glass of freshly squeezed carrot juice daily.
Gingko biloba. Pour 50 g of dry crushed leaves of this tree with 0.5 liters of alcohol. Infuse for three weeks in a dark place. Drink 10 drops of the remedy each time before meals.

Prevention

Currently, specific methods for the prevention of this disease have not been developed due to the lack of sufficient information about the causes of its occurrence. It is important to follow the general recommendations:

Lead a healthy lifestyle, giving up bad habits.
Eat properly and nutritiously.
Avoid contact with radiation and harmful substances.
Regularly undergo preventive examinations.

Erythrocytosis in men

According to medical statistics, polycythemia is slightly more common in men than in women. The ratio is approximately 1.2:1. Erythrocytosis in women and men is diagnosed at an average of 60 years. At a young age, the disease is very rare.

In children, physiological erythrocytosis can be diagnosed immediately after birth. In neonates, this diagnosis is confirmed if venous hematocrit increases by ≥ 65%.

Polycythemia in newborns can manifest as severe symptoms (thrombosis, heart failure, CNS disorders) or mild (lethargy, tremor, elevation). Sometimes the skin of babies is too ruddy or darkish. There may be poor appetite. But in most cases, newborns with polycythemia do not have severe symptoms.

Most often, polycythemia develops in post-term children or in babies whose mothers suffered. hypertension, smoked during pregnancy, etc. This condition may also occur due to the fact that the child received too much blood from the placenta during fetal development. Probably its manifestation against the background of congenital defects, hypoxia and etc.

For the purpose of treatment, intravenous fluid replacement is carried out, sometimes - partial exchange transfusion. This allows you to reduce the concentration of red blood cells.

Diet for polycythemia

For people who suffer from erythremia, it is advisable to minimize the amount of protein foods on the menu. The fact is that protein contributes to the stimulation of the hematopoietic system. It is especially important to stop consuming fatty fish and meats, as well as hard cheeses with a high fat content. An increase in indicators should not be allowed, as this is dangerous in terms of thrombosis.

vegetables - raw, stewed, boiled;
fresh fruits and berries;
fermented milk products - yogurt, kefir, curdled milk, fermented baked milk, low-fat cottage cheese and sour cream;
greens - parsley, dill;
eggs;
dried fruits;
whole grain dishes;
green tea with ginger;
seaweed;
bitter chocolate;
nuts.

Consequences and complications

The following conditions may develop as complications:

Blood clots. As blood density increases, blood flow slows down. In addition, there are changes in platelets, and this leads to an increased risk of blood clots. In turn, thrombosis increases the likelihood of heart attack, stroke, thromboembolism, deep vein thrombosis.
Splenomegaly- enlargement of the spleen. Since the spleen with erythremia works very intensively, as a result, the organ increases.
open ulcers of the stomach, esophagus, duodenum.
- inflammation of the joints.
gallstone And urolithiasis disease - a consequence of an increase in the concentration of uric acid. Myelofibrosis progressive replacement of bone marrow with scar tissue.
myelodysplastic syndrome- Immaturity or malfunction of stem cells.
Spicy- malignant process.

Forecast

If a patient is diagnosed with polycythemia vera, the prognosis depends on the degree of development of the disease. In the absence of adequate treatment, a third of patients die within the first five years. The most common cause of death is thrombosis and. In some patients with the third stage of polycythemia (20-50%), the disease transforms into acute leukemia.

If the correct therapy is carried out for a patient diagnosed with erythremia, the prognosis for life is more optimistic. Such patients manage to prolong life for decades.

List of sources

Guseva S. A., Bessmeltsev S. S., Abdulkadyrov K. M., Goncharov Ya. P. Polycythemia vera. - Kyiv, St. Petersburg: Logos, 2009. - 405 p.
Demidova A. V., Kotsyubinsky N. N., Mazurov V. I. Erythremia and secondary erythrocytosis. - St. Petersburg: Publishing house of SPbMAPO, 2001. - 228 p.
Demidova A. V., Khoroshko N. D. Erythremia and erythrocytosis. In the book: Vorobyov A. I. (ed.). Guide to hematology. Moscow: Newdiamed; 2003; v. 2: 21-27

Polycythemia vera (erythremia, Wakez's disease, polycythemia erythema) - PV - a chronic neoplastic myeloproliferative disease with stem cell damage, proliferation of three hematopoietic lineages, increased production of red blood cells and, to a lesser extent, white blood cells and platelets. At a certain stage of the disease, myeloid metaplasia of the spleen joins.

The incidence of polycythemia vera is approximately 1 random per 100,000 population per year and has a clear upward trend in recent years. Men get sick a little more often than women (1.2: 1). The average age of patients is 60 years old, patients under 40 years old make up only 5%.

Etiopathogenesis. Polycythemia vera is a clonal neoplastic disease, which is based on the transformation of the hematopoietic stem cell. Since malignant transformation occurs at the level of a pluripotent stem cell, all three hematopoietic lineages are involved in the process. In patients suffering from PV, there is an increased content of CFU-GEMM (colony-forming units - granulocytic, erythroid, macrophage and megakaryocytic) - precursor cells close to the pluripotent stem cell. In cell culture, these cells actively proliferate in the absence of erythropoietin. A low serum erythropoietin level is a specific feature of PV. In the bone marrow, hyperplasia is observed predominantly of erythroid cells, as well as granulocytic and megakaryocytic sprouts. A characteristic feature is the presence of clusters of polymorphic megakaryocytes (from small to giant). Myelofibrosis is rarely observed at the time of diagnosis, but manifests itself distinctly with a long course of the disease. Gradually there is an increase in the number of reticulin and collagen fibers, myelofibrosis develops and myelopoiesis is reduced. The mass of circulating erythrocytes (MCE) increases, hematocrit rises, blood viscosity increases (there is a significant increase in the content of hemoglobin in the blood (from 180 g / l and above), erythrocytes (from 6.6 x 10 12 / l) and the hematocrit index (from 55 % and above). These factors, along with thrombocytosis, lead to impaired microcirculation and thromboembolic complications. In parallel, myeloid metaplasia of the spleen joins. In PV, there is no specific cytogenetic marker; anomalies.

Clinical picture changes with the course of the disease and is determined mainly by the stage of the disease. In the domestic literature, it is customary to distinguish four stages of PV, which reflect the pathological processes occurring in the bone marrow and spleen of patients.

Stages:

I - initial, asymptomatic (5 years or more):

spleen is not palpable

moderate erythrocytosis

moderate plethora

panmyelosis in the bone marrow

vascular and thrombotic complications are possible but not common

External manifestations of the disease - plethora, acrocyanosis, erythromelalgia (burning pain, paresthesia in the fingertips) and skin itching after washing. An increase in MCE and, consequently, in the volume of circulating blood leads to arterial hypertension. If the patient has previously suffered from hypertension, then there is an increase in blood pressure, antihypertensive therapy becomes ineffective. The manifestations of coronary heart disease, cerebral atherosclerosis are aggravated. Since MCE increases gradually, plethora, an increase in the number of erythrocytes and hemoglobin, signs of microcirculation disorders in a number of patients appear 2-4 years before the diagnosis is made.

II - erythremic, deployed (10-15 years):

A. Without myeloid metaplasia of the spleen

general condition is disturbed

severe plethora (Hb 200 g/l or more)

thrombotic complications (stroke, myocardial infarction, necrosis of the fingertips)

panmyelosis

erythromelalgia (pain in limbs and bones)

In the picture of peripheral blood, in addition to erythrocytosis, neutrophilia is often present with a shift of the leukocyte formula to the left to single myelocytes, as well as basophilia and thrombocytosis. In the bone marrow, total three-growth hyperplasia with pronounced megakaryocytosis is detected, and reticulin myelofibrosis is possible. But at this stage of the disease, myeloid metaplasia of the spleen (MMS) is still absent, and the observed splenomegaly is due to increased sequestration of erythrocytes and platelets. Vascular complications are more frequent and severe than in the first stage of the disease. In the pathogenesis of thrombosis, an important role is played by an increase in MCE, which leads to an increase in blood viscosity and a slowdown in blood flow, thrombocytosis, and endothelial dysfunction. Ischemia associated with impaired arterial blood flow occurs in 24-43% of patients. Thrombosis of the vessels of the brain, coronary and blood-supplying organs of the abdominal cavity arteries predominate. Venous thrombosis occurs in 25-30% of patients and is the cause of death in about a third of patients with PV. Frequent thrombosis of the veins of the portal system and mesenteric veins. In a number of patients, it is thrombotic complications that become a manifestation of PV. Polycythemia vera may be accompanied by hemorrhagic syndrome: frequent nosebleeds and bleeding after tooth extraction. Hypocoagulation is based on a slowdown in the conversion of fibrinogen to fibrin, which occurs in proportion to an increase in hematocrit, and a violation of blood clot retraction. Erosions and ulcers of the stomach and duodenum are considered as visceral complications of PV.

B. With myeloid metaplasia of the spleen (MMS).

hepatosplenomegaly

plethora is moderately expressed

panmyelosis

increased bleeding

thrombotic complications

Splenomegaly increases, the number of leukocytes increases, the shift of the leukocyte formula to the left becomes more pronounced. In the bone marrow - panmyelosis; gradually develops reticulin and focal collagen myelofibrosis. The number of erythrocytes and platelets is somewhat reduced due to their increased destruction in the spleen, as well as the gradual replacement of hematopoietic tissue with fibrous tissue. At this stage, stabilization of the condition of patients can be observed, the level of hemoglobin, erythrocytes and platelets approaches the norm without therapeutic measures.

III - anemic:

anemic s-m (even pancytopenia)

severe myelofibrosis

liver, spleen enlarged

In the bone marrow, collagen myelofibrosis increases and myelopoiesis is reduced. The hemogram shows anemia, thrombocytopenia, pancytopenia. IN clinical picture diseases, anemic and hemorrhagic syndromes may be present, splenomegaly and cachexia are increasing. The outcome of the disease can be transformation into acute leukemia and myelodysplastic syndrome (MDS).

Diagnostics. Currently, the criteria developed by the American Polycythemia Vera Study Group (PVSG) are used to establish the diagnosis of polycythemia vera. You-

1) an increase in the mass of circulating erythrocytes (more than 36 ml / kg for men and more than 32 ml / kg for women);

2) normal saturation of arterial blood with oxygen (pO2 over 92%);

3) splenomegaly.

1) thrombocytosis (platelet count over 400 x 10 9 /l);

2) leukocytosis (the number of leukocytes is more than 12 x 10 9 / without signs of infection);

3) alkaline phosphatase activity (neutrophils above 100 units in the absence of fever or infection);

4) high content of vitamin B12 (more than 900 pg/ml).

The diagnosis of PV is considered reliable if the patient has all three signs of category A, or if the first and second signs of category A and any two signs of category B are present.

Currently, the characteristic histological picture of the bone marrow is considered the most important diagnostic sign; hyperplasia of cells of erythroid, granulocytic and megakaryocytic sprouts with a predominance of erythroid, accumulations of polymorphic megakaryocytes (from small to giant). Myelofibrosis is rarely observed at the time of diagnosis, but becomes distinct with a long course of the disease.

At stage I, true polycythemia, characterized by isolated erythrocytosis, must be differentiated from secondary erythrocytosis, which is a response to any pathological process in the body and can be both true and relative.

Relative erythrocytosis is a consequence of hemoconcentration, that is, MCE is normal, but the plasma volume is reduced, which is observed when the body is dehydrated (for example, taking diuretics, polyuria in patients with diabetes mellitus, vomiting and diarrhea), loss of a large amount of plasma during burns.

True secondary erythrocytosis (MCE is increased, hematocrit is increased) is due to increased production of erythropoietin. The latter is compensatory in nature and is caused by tissue hypoxia in people living at a significant altitude above sea level, in patients with pathology of the cardiovascular and respiratory systems, and in smokers. This category also includes patients with hereditary hemoglobinopathies, characterized by an increased affinity of hemoglobin for oxygen, which is released in the tissues of the body in a smaller amount. Inadequate production of erythropoietin is observed in kidney diseases (hydronephrosis, vascular pathology, cysts, tumors, congenital anomalies), hepatocellular carcinoma, large uterine myoma. An essential differential diagnostic sign is the level of erythropoietin in blood serum.

Treatment. In the initial stages of the disease, it is recommended to use bloodletting, which greatly alleviates the manifestations of the plethoric syndrome. The method of choice for lowering hematocrit (and hemoglobin to normal values) is phlebotomy (exfusion), which is recommended if the hematocrit exceeds 0.54. The goal of treatment is a hematocrit of less than 0.42 for women and 0.45 for men. In modern conditions, bloodletting can be replaced by erythrocytapheresis. In addition, to facilitate bloodletting and prevent thrombotic complications, patients are given courses of antiplatelet therapy (aspirin, reopoliglyukin, etc.). The choice of a treatment method in advanced stage II of PV is perhaps the most difficult task. In addition to erythrocytosis, patients have leukocytosis and thrombocytosis, and the latter can reach very high numbers. Some patients have already experienced any thrombotic complications, and exfusions increase the risk of thrombosis.

When individualizing therapy, the age of patients should be taken into account. So the treatment of patients younger than 50 years, without a history of thrombotic complications and severe hyperthrombocytosis (< 1000,0 х 10 9 /л) может быть ограничено только кровопусканиями в сочетании с терапией аспирином (или без него) в дозе 100-375 мг в день.

Patients over 70 years of age with a history of thrombotic complications and severe hyperthrombocytosis are treated with myelosuppressive drugs. Patients aged 50–70 years without thrombotic complications and severe hyperthrombocytosis may be treated with myelosuppressive agents or phlebotomy, although the latter may increase the risk of thrombotic complications.

At present, in addition to bloodletting and antiplatelet agents, hydroxyurea and alpha-interferon are mainly used for the treatment of PV, less often busulfan, and anagrelide is used abroad. Hydroxyurea may be the drug of choice if patients with PV have severe leukocytosis and thrombocytosis. But for young patients, the use of hydroxyurea is limited by its mutagenic and leucosogenic effects. In addition to hydroxyurea, interferon-alpha is widely used in the treatment of PV. Firstly, IF-a suppresses pathological proliferation well and does not have a leukemogenic effect. Secondly, like hydroxyurea, it significantly reduces the production of platelets and leukocytes. The ability of IF-a to eliminate itching caused by taking water procedures deserves special attention.

aspirin in daily dose 50-250 mg, as a rule, eliminates microcirculation disorders. The use of this drug or other antiplatelet agents for therapeutic or prophylactic purposes is recommended for all patients with PV.

Unfortunately, there is currently no effective treatment for III anemic stage of IP. Therapy is limited to palliative care. Anemic and hemorrhagic syndrome is corrected by transfusions of blood components. The effectiveness of hematopoietic stem cell transplantation in patients with PV in the stage of myelofibrosis with splenomegaly and pancytopenia and transformation into acute leukemia or MDS has been reported. Three-year survival of patients after transplantation was 64%.

Forecast. Despite a long and in some cases favorable course, PV is a serious disease and is fraught with fatal complications that reduce the life expectancy of patients. The most common cause of death in patients is thrombosis and embolism (30-40%). In 20-50% of patients in the stage of post-polycythemic myelofibrosis (stage III PV), a transformation into acute leukemia occurs, which has an unfavorable prognosis - a three-year survival rate of only 30%.