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

Features of the disease

Primary (true) polycythemia is a blood disease from the leukemia group that occurs idiopathically (without visible reasons), proceeds for a long time (chronically) and is characterized by an increase in the number of red blood cells, an increase in hematocrit and blood viscosity. Synonyms for the name of the pathology are Vaquez-Osler disease, erythremia, primary erythrocytosis. The consequences of erythrocytosis and blood thickening in this myeloproliferative disease can be serious and relate to the risk of thrombosis, an increase in size and disruption of the spleen, an increase in the volume of circulating blood, etc.

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

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

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

Causes of pathology

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

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

As for the risk factors for the development of polycythemia vera, presumably they may be the following:

• lung diseases;
• long stay at high altitudes above sea level;
• pulmonary hypoventilation syndromes;
• various hemoglobinopathies;
• long history of smoking;
• tumors of bone marrow, blood;
• hemoconcentration with long-term use of diuretics;
• burns of a large part of the body;
• severe stress;
• diarrhea;
• exposure to x-rays, radiation;
• poisoning by chemical vapors, penetration through the skin;
• entry of toxic substances into the gastrointestinal tract;
• treatment with gold salts;
• advanced tuberculosis;
• major surgical interventions;
• “blue” heart defects;
• kidney pathologies - hydronephrosis, stenosis of the renal arteries.

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

Classification of polycythemia vera

The disease is classified into the following stages:

1. The first, or initial stage. It can last more than 5 years and represents the development of plethoric syndrome, that is, increased blood supply to organs. At this stage, symptoms may be moderate, and no complications arise. A general blood test reflects a slight increase in the number of red blood cells, a bone marrow puncture shows an increase in erythropoiesis or the production of all the main elements of blood, with the exception of lymphocytes.
2. The second is stage A, or polycythemic stage. Duration - from 5 to 15 years. Plethoric syndrome is more pronounced, an enlargement of the spleen and liver is observed ( hematopoietic organs), thrombus formation in veins and arteries is often recorded. No tumor growth was observed in the peritoneal organs. If this stage ends with a decrease in the number of platelets - thrombocytopenia, then the patient may experience various bleeding. Frequent hemorrhages cause a lack of iron in the body. A general blood test reflects an increase in red blood cells, platelets, and leukocytes; in advanced cases, a decrease in platelets. The myelogram shows increased formation of most blood cells (with the exception of lymphocytes), the formation of scar changes brain
3. The second is stage B, or polycythemic stage with myeloid metaplasia of the organ - the spleen. The patient's spleen and often the liver continue to increase in size. Puncture of the spleen reveals tumor growth. Frequent thromboses interspersed with bleeding are observed. In the general analysis, there is an even greater increase in the number of erythrocytes, leukocytes, there are erythrocytes of different sizes, shapes, and immature precursors of all blood cells are present. The number of scar changes in the bone marrow increases.
4. Third, or anemic stage. It is the outcome of a disease in which the activity of blood cells is depleted. The number of red blood cells, white blood cells, and platelets is greatly reduced, the liver and spleen are enlarged with myeloid metaplasia, and extensive scarring occurs in the bone marrow. A person becomes disabled, most often due to the consequences of thrombosis or the addition of acute leukemia, myelofibrosis, hematopoietic hypoplasia or chronic myeloid leukemia. This stage is registered approximately 10-20 years after the development of the pathology.

Symptoms of manifestation

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

1. Change in skin tone, dilation of veins. Most often, in the neck area of ​​an adult, the veins begin to become very visible; their pattern becomes stronger due to swelling and overfilling with blood. But the skin signs become the most obvious: the skin color becomes dark red, literally cherry. This is most noticeable in the neck, arms, and face, which is associated with overfilling of the subcutaneous arteries with blood. At the same time, many patients mistakenly think that blood pressure rises due to hypertension, and therefore often continue to take blood pressure medications and do not consult a doctor. If you pay close attention to your health, you will notice that your lips and tongue have also changed their color and become red-blue. The blood vessels of the eyes also become engorged, their plethora leads to hyperemia of the sclera and conjunctiva of the organs of vision. The hard palate remains the same color, but soft palate also becomes brighter, burgundy.
2. Itchy skin. All described changes in the skin in approximately half of the cases are complemented by severe discomfort and itching. This symptom is very characteristic of erythremia, both primary and secondary. Since after acceptance water procedures In patients, histamine is released, as well as prostaglandins, and the itching of the skin may become even more pronounced after a bath or shower.
3. Pain in the limbs. Many people develop obliterating endarteritis, which results in persistent and strong painful sensations in the legs. They can intensify with exercise, long walking, in the evening, and at first they are often perceived as a symptom of fatigue in an elderly person. Pain is also observed with palpation and tapping of flat bones, which reflects the process of hyperplasia and cicatricial changes in the bone marrow. Next view pain in a person with polycythemia vera - persistent burning pain in the area of ​​​​large and small joints of the legs, which resemble gouty pain and are caused by the same reason as gout - an increase in the level of uric acid. Another type of pain is severe, poorly tolerated pain in the fingers and toes, in which the skin becomes bluish-red and marks appear on it. blue spots. These pains are caused by an increase in the number of platelets and the appearance of capillary microthrombosis.
4. Splenomegaly. An increase in spleen size is observed in almost every person with polycythemia vera, but different stages diseases. This occurs due to increased filling of the spleen with blood and the development of myeloproliferative phenomena. Somewhat less frequently, but still occurring, is a strong increase in the size of the liver - hepatomegaly.
5. Peptic ulcer disease. About one in ten people with Vaquez-Osler disease develop ulcers in the small intestine (usually in the duodenum) and in the stomach. This is due to the activation Helicobacter bacteria pylori, as well as the development of microthrombosis in the gastrointestinal tract.
6. Thrombosis and bleeding. Almost all patients at a certain stage develop a tendency to thrombosis, and until recently, patients died from such complications at an early stage of the disease. Currently underway modern treatment can prevent the appearance of blood clots in the brain, spleen, legs, which threaten embolism and death. Increased blood viscosity characterizes polycythemia vera in initial stages, and later, against the background of depletion of the platelet formation system, bleeding develops - it is observed in the gums, nose, uterus, and gastrointestinal tract.

There are other signs of polycythemia vera that a person may complain about, but they are not very specific and can be characteristic of different pathologies:

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

In general, the disease is characterized by a long and sometimes benign course, especially with adequate treatment. But some people, especially those not receiving therapy, may experience early onset of various effects of polycythemia vera.

Possible complications

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

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

Carrying out diagnostics

Make a diagnosis of this disease not easy, especially in the absence of a characteristic clinical picture and in the presence of only common symptoms. However, the totality of hematological and biochemical tests, as well as some distinctive features of the patient’s appearance, coupled with his complaints, will help the doctor determine the cause of the changes occurring.

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

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

1. Moderate or severe thrombocytosis (above 400*10*9 l), neutrophilic leukocytosis (above 12*10*9 l) with the presence increased amount basophils and eosinophils.
2. Increased reticulocyte count.
3. The appearance of myelocytes and metamyelocytes in the blood.
4. Increases blood viscosity by 500-800%.
5. Severe decrease in ESR.
6. Increase in the mass of circulating red blood cells.
7. Promotion alkaline phosphatase, vitamin B12 in serum.
8. Increase in the amount of uric acid in the serum.
9. Blood saturation in the arteries with oxygen is above 92%.
10. The appearance of colonies of erythrocytes in a test tube.
11. Decrease in erythropoietin levels.
12. Change color index, being less than 1.

At the stage of myelofibrosis, hemoglobin and red blood cell levels may return to normal, but at the same time the number of leukocytes increases greatly, their immature forms appear, and the presence of erythroblasts is diagnosed. As for the myelogram, which is obtained by puncturing the bone marrow, the following changes are revealed:

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

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

1. Hepatosplenomegaly.
2. Tendency to thrombosis.
3. Increased sweating combined with weight loss and weakness.
4. Presence of gene abnormalities if genetic testing was performed when we're talking about about primary erythremia.
5. Increasing the average amount of circulating blood.

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

Treatment methods

The sooner a person seeks help, the more effective therapy can be. At the third stage, or when another tumor process is superimposed on erythremia, symptomatic therapy is carried out in combination with treatment with chemotherapy. Chemotherapy treatment may be recommended at other stages of the disease, but the body does not always respond adequately to it. Among the symptomatic remedies that improve the quality of life, the following are used:

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

Treatment methods for polycythemia vera may include:

1. Bloodletting, or removing a small amount of blood from the bloodstream (phlebotomy). As a rule, they are done in a volume of 100-400 ml (according to indications) and a break of 3-4 days in a course of several sessions. After such manipulations, the blood becomes more fluid, but they cannot be done if there is a recent history of blood clots. Before treatment with bloodletting, the patient is administered a solution of Reopoliglucin, as well as Heparin.
2. Erythrocytapheresis. Used to cleanse the blood of excess red blood cells, as well as platelets. Such sessions are done once a week.
3. Chemotherapy. It is used, as a rule, when the disease reaches the tumor stage - second B. Other indications for chemotherapy are the presence of complications from the peritoneal organs, the general difficult situation of the person, and an increase in the amount of all blood elements. For chemotherapy or cytoreductive therapy, cytostatics, antimetabolites, alkylating drugs, and biological drugs are used. The most commonly prescribed medications are Leukeran, Hydroxyurea, Myelosan, and recombinant interferon.
4. Treatment of iron deficiency with androgens, erythropoietin, which are most often used in combination with glucocorticosteroids.
5. Radiation therapy. It is used to irradiate the area of ​​the spleen and stop the cancer process in it; it is used when the organ greatly increases in size.
6. Transfusion of red blood cells from purified red blood cells. Used for severe anemia to the point of coma. If thrombocytopenia increases in the final stages of polycythemia vera, a transfusion of platelet mass from a donor may be necessary.

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

Features of treatment in pregnant women

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

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

What not to do

It is strictly forbidden to use diuretics, which further thicken the blood. Also in our time, the use of radioactive phosphorus preparations, which seriously inhibit myelopoiesis and often lead to the development of leukemia, is limited. You also cannot maintain the same nutritional system: the diet must change. All foods that enhance hematopoiesis, such as liver, are prohibited. It is better to create a dairy-vegetable diet and avoid excess meat.

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

Prevention and prognosis

Prevention methods have not yet been developed. The prognosis for life varies depending on the severity of the disease. Without treatment, up to a third of patients die within the first 5 years from diagnosis. If you carry out full-fledged therapy, you can extend a person’s life to 10-15 years or more. The most common cause of death is thrombosis, and only occasionally do people die from blood cancer (leukemia) or severe bleeding.

Polycythemia is a disease that can be identified just by looking at a person's face. And if you still diagnostic examination, then there will be no doubt at all. In the medical literature you can find other names for this pathology: erythremia, Vaquez disease. Regardless of the term chosen, the disease poses a serious threat to human life. In this article we will talk in more detail about the mechanism of its occurrence, primary symptoms, stages and proposed treatment methods.

General information

Polycythemia vera is a myeloproliferative blood cancer that produces red blood cells in excess quantities. To a lesser extent, an increase in other enzyme elements, namely leukocytes and platelets, is observed.

Red blood cells (aka erythrocytes) supply oxygen to all cells human body, delivering it from the lungs to the internal organ systems. They are also responsible for removing carbon dioxide from tissues and transporting it to the lungs for later exhalation.

Red blood cells are continuously produced in the bone marrow. It is a collection of sponge-like tissues, localized inside the bones and responsible for the process of hematopoiesis.

Leukocytes are white blood cells that help fight various infections. Platelets are fragments that are activated when the integrity of blood vessels is disrupted. They have the ability to stick to each other and clog the hole, thereby stopping bleeding.

Polycythemia vera is characterized by excess production of red blood cells.

Prevalence of the disease

This pathology, as a rule, is diagnosed in adult patients, but can occur in adolescents and children. For a long time, the disease may not make itself felt, that is, it may be asymptomatic. According to studies, the average age of patients varies from 60 to approximately 79 years. Young people get sick much less often, but their illness is much more severe. According to statistical data, representatives of the stronger sex are diagnosed with polycythemia several times more often.

Pathogenesis

Most of the health problems associated with this disease arise from the continuous increase in the number of red blood cells. As a result, the blood becomes excessively thick.

On the other hand, its increased viscosity provokes the formation of clots (thrombi). They can interfere with normal blood flow through arteries and veins. This situation often causes strokes and heart attacks. The whole point is that thick blood flows several times slower through the vessels. The heart has to make more efforts to literally push it through.

Slowing blood flow does not allow internal organs to receive the required amount of oxygen. This entails the development of heart failure, headaches, angina, weakness and other health problems that are not recommended to be ignored.

Classification of the disease

• I. Initial stage.

1. Lasts from 5 years or more.
2. The spleen is of normal size.
3. Blood tests show a moderate increase in the number of red blood cells.
4. Complications are diagnosed extremely rarely.

• II A. Polycythemic stage.

1. Duration from 5 to approximately 15 years.
2. There is an increase in some organs (spleen, liver), bleeding and thrombosis.
3. There are no areas in the spleen itself.
4. Bleeding can cause iron deficiency in the body.
5. The blood test shows a persistent increase in red blood cells, white blood cells and platelets.

• II B. Polycythemic stage with myeloid metaplasia of the spleen.

1. Analyzes show increased content all blood cells except lymphocytes.
2. A tumor process is observed in the spleen.
3. IN clinical picture exhaustion, thrombosis, and bleeding appear.
4. Gradual scar formation occurs in the bone marrow.

• III. Anemic stage.

1. Happens in the blood sharp decline erythrocytes, platelets and leukocytes.
2. There is a marked increase in the size of the spleen and liver.
3. This stage usually develops 20 years after confirmation of the diagnosis.
4. The disease can transform into acute or chronic leukemia.

Causes of the disease

Unfortunately, at present experts cannot say which factors lead to the development of a disease such as polycythemia vera.

Most are inclined to the viral-genetic theory. According to her, special viruses(there are about 15 of them in total) are introduced into the human body and under the influence of some factors that negatively affect immune protection, penetrate into the cells of the bone marrow and lymph nodes. Then, instead of maturing as expected, these cells begin to rapidly divide and multiply, forming more and more new fragments.

On the other hand, the cause of polycythemia may lie in a hereditary predisposition. Scientists have proven that close relatives of the sick person, as well as people with abnormal chromosome structure, are more susceptible to this disease.

Factors predisposing to the onset of the disease

• X-ray exposure, ionizing radiation.
• Intestinal infections.
• Viruses.
• Tuberculosis.
• Surgical interventions.
• Frequent stress.
• Long-term use of certain groups of medications.

Clinical picture

Starting from the second stage of development of the disease, literally all systems of internal organs are drawn into the pathological process. Below we list subjective feelings sick.

• Weakness and a persistent feeling of fatigue.
• Increased sweating.
• Noticeable decrease in performance.
• Severe headaches.
• Memory impairment.

Polycythemia vera may also be accompanied by the following symptoms. In each specific case, their severity varies.

Diagnostics

First of all, the doctor collects a complete medical history. He may ask a number of clarifying questions: when exactly did the malaise/shortness of breath/painful discomfort appear, etc. It is equally important to determine the presence of chronic ailments, bad habits, possible contacts with toxic substances.

A physical examination is then performed. The specialist determines the color of the skin. By palpation and tapping, an enlarged spleen or liver is detected.

To confirm the disease in mandatory blood tests are prescribed. If the patient has this pathology, the test results may be as follows:

• Increase in the number of red blood cells.
• Elevated hematocrit parameters (percentage of red blood cells).
• High hemoglobin levels.
• Low erythropoietin levels. This hormone is responsible for stimulating the bone marrow to produce new red blood cells.

Diagnosis also involves brain aspiration and biopsy. The first version of the study involves taking the liquid part of the brain, and a biopsy - the solid component.

Polycythemia disease is confirmed by gene mutation tests.

What should the treatment be?

It is not possible to completely overcome a disease such as polycythemia vera. That is why therapy focuses exclusively on reducing clinical manifestations and reducing thrombotic complications.

Patients are first prescribed bloodletting. This procedure implies the removal of a small amount of blood (from 200 to approximately 400 ml) from therapeutic purpose. It is necessary to normalize the quantitative parameters of the blood and reduce its viscosity.

Patients are usually prescribed Aspirin to reduce the risk of developing various types of thrombotic complications.

Chemotherapy is used to maintain a normal hematocrit when severe pruritus or increased thrombocytosis occurs.

Bone marrow transplantation for this disease is performed extremely rarely, since this pathology is not fatal if adequately treated.

It should be noted that the specific treatment regimen is selected individually in each case. The therapy described above is for informational purposes only. It is not recommended to try to cope with this disease on your own.

Possible complications

This disease is quite serious, so its treatment should not be neglected. Otherwise, the likelihood of unpleasant complications increases. These include the following:

Forecast

Vaquez disease is a rare disease. Symptoms that appear in the early stages of its development should be the reason for immediate examination and subsequent therapy. In the absence of adequate treatment, if the disease is not diagnosed in a timely manner, death occurs. The main cause of death is most often vascular complications or transformation of the disease into chronic leukemia. However, competent therapy and strict adherence to all recommendations from the doctor can significantly extend the patient’s life (by 15-20 years).

We hope that all the information presented in this article will be truly useful to you. Be healthy!

Polycythemia is a chronic disease in which the amount of red blood cells (erythrocytes) in the blood increases. Also, with this pathology, in 70% of patients the number of platelets and leukocytes changes upward.

The disease is not very common - no more than five cases are registered annually per one million people. Most often, polycythemia disease develops in middle-aged and elderly people. According to statistics, males suffer from this pathology five times more often than women. Today we will take a closer look at a condition such as polycythemia; the symptoms and treatment of the pathology will be described below.

Reasons for the development of the disease

Polycythemia is not a malignant disease. To date, the exact causes of the disease are unknown. It is believed that the development of pathology is caused by a mutation of a special enzyme in the bone marrow. Gene changes lead to excessive division and growth of all blood cells, and especially red blood cells.

Classification of the disease

There are two groups of the disease:

True polycythemia, or Vaquez's disease, which in turn is divided into primary (that is, acts as an independent disease) and secondary (secondary polycythemia develops due to chronic lung diseases, tumors, hydronephrosis, and elevation).

Relative polycythemia (stress or false) - with this state the level of red blood cells remains within normal limits.

Polycythemia: symptoms of the disease

Very often the disease is asymptomatic. Sometimes, as a result of an examination for completely different reasons, polycythemia vera may be accidentally detected. We will consider the symptoms that you should pay attention to below.

Dilatation of saphenous veins

With polycythemia, dilated saphenous veins appear on the skin, most often in the neck area. With this pathology, the skin becomes reddish-cherry in color, this is especially noticeable on open areas of the body - neck, hands, face. The mucous membrane of the lips and tongue has a bluish-red color, the whites of the eyes seem to be bloodshot.

Such changes are caused by the overflow of blood, rich in erythrocytes, of all superficial vessels and slowing down its rheological properties(speed of movement), as a result of which the main part of hemoglobin (red pigment) goes into a reduced form (that is, undergoes chemical changes) and changes color.

Itchy skin

Almost half of patients suffering from polycythemia experience severe itchy skin, especially evident after adoption warm bath. This phenomenon acts as specific sign vera polycythemia. Itching occurs due to release active substances into the blood, in particular histamine, which is able to expand the skin capillaries, which leads to increased blood circulation in them and the appearance of specific sensations.

Erythromelalagia

This phenomenon is characterized by short-term severe pain in the area of ​​the fingertips. They are provoked by an increase in the level of platelets in the small vessels of the hand, as a result of which numerous microthrombi are formed, clogging the arterioles and blocking the flow of blood to the tissues of the fingers. External signs of this condition are redness and the appearance of bluish spots on the skin. For this purpose, it is recommended to take aspirin.

Splenomegaly (enlarged spleen)

In addition to the spleen, the liver, or rather its size, may also change. These organs are directly involved in the formation and destruction of blood cells. An increase in the concentration of the latter leads to an increase in the size of the liver and spleen.

and stomach

So serious surgical pathology develops due to thrombosis small vessels mucous membrane digestive tract. The acute result is necrosis (necrosis) of a section of the organ wall and the formation of an ulcerative defect in its place. In addition, the stomach's resistance to Helicobacter (a microorganism causing gastritis and ulcers).

Blood clots in large vessels

The veins of the lower extremities are more susceptible to this pathology. from the vessel wall, can, bypassing the heart, penetrate into the pulmonary circulation (lungs) and provoke PE (thromboembolism pulmonary artery) - a condition incompatible with life.

Bleeding gums

Despite the fact that the number of platelets in the peripheral blood changes and its coagulability increases, gingival bleeding may occur with polycythemia.

Gout

When the level of uric acid increases, its salts are deposited in various joints and provoke a sharp pain syndrome.

• Pain in the limbs. This symptom causes damage to the arteries of the legs, their narrowing and, as a result, poor circulation. This pathology is called “obliterating endarteritis”
• Pain in flat bones. Increased activity bone marrow (the place of development of blood cells) provokes sensitivity of flat bones to mechanical influences.

Deterioration of the general condition of the body

With a disease such as polycythemia, the symptoms may be similar to signs of other pathologies (for example, anemia): headaches, constant fatigue, tinnitus, dizziness, flickering “goosebumps” before the eyes, shortness of breath. An increase in the viscosity of the blood activates the compensatory vascular response , as a result, there is an increase blood pressure. With this pathology, complications are often observed in the form of heart failure and microcardiosclerosis (replacement of the muscle tissue of the heart with connective tissue that fills the defect, but does not perform the necessary functions).

Diagnostics

Polycythemia is detected by the results of a general blood test, which reveals:

increased number of red cells from 6.5 to 7.5.10^12/l;

increased hemoglobin level - up to 240 g/l;

the total volume of erythrocytes (TEV) exceeds 52%.

Since the number of red blood cells cannot be calculated based on measurements of the above values, radionuclide diagnostics are used for measurement. If the mass of red blood cells exceeds 36 ml/kg in men and 32 ml/kg in women, then this reliably indicates the presence of Vaquez disease.

With polycythemia, the morphology of red blood cells is preserved, that is, they do not change their normal shape and size. However, with the development of anemia as a result of increased bleeding or frequent bloodletting, microcytosis (a decrease in red blood cells) is observed.

Polycythemia: treatment

Bloodletting has a good therapeutic effect. It is recommended to remove 200-300 ml of blood weekly until the TEC level drops to the desired value. If there are contraindications for bloodletting, the percentage of red blood cells can be restored by diluting the blood by adding a liquid part to it (high molecular weight solutions are administered intravenously).

It should be borne in mind that quite often bloodletting leads to the development iron deficiency anemia, in which corresponding symptoms and an increase in platelet counts are observed.

For a disease such as polycythemia vera, treatment involves following a certain diet. It is recommended to limit the consumption of meat and fish products, as they contain high quantity a protein that actively stimulates the activity of the hematopoietic organs. You should also avoid fatty foods. Cholesterol contributes to the development of atherosclerosis, resulting in blood clots, which are already formed in large quantities in people suffering from polycythemia.

Also, if polycythemia is diagnosed, treatment may include chemotherapy. It is used for increased thrombocytosis and severe itching. As a rule, this is a “cytoreductive agent” (the drug “Hydroxycarbamide”).

Until recently, injections of radioactive isotopes (usually phosphorus-32) were used to suppress bone marrow. Today, such treatment is increasingly being refused due to high speed leukemic transformation.

Therapy also includes injections of interferon; in the treatment of secondary thrombocytosis, the drug Anagrelide is used.

For this pathology, it is very rarely performed since polycythemia is a disease that is not fatal, provided, of course, adequate treatment and constant monitoring.

Polycythemia in newborns

Polycythemia is a pathology, the symptoms of which can be found in newborns. This disease is a response of the baby’s body to hypoxia, which could have been provoked. To correct hypoxia, the baby’s body begins to synthesize a large number of red blood cells.

In addition to the respiratory condition, newborns can also develop polycythemia vera. Twins are especially at risk of this risk.

Polycythemia in a newborn develops in the first weeks of life, its first manifestations are an increase in hematocrit (up to 60%) and a significant increase in hemoglobin levels.

Neonatal polycythemia has several stages: initial, proliferation and depletion. Let's briefly describe them.

The initial stage of the disease has virtually no clinical manifestations. Polycythemia in a child can be detected at this stage only by examining peripheral blood parameters: hematocrit, hemoglobin and red blood cell level.

At the stage of proliferation, an enlargement of the liver and spleen develops. Plethoric phenomena are observed: skin acquire a characteristic “plethoric-red” hue, the child shows anxiety when touching the skin. Plethoric syndrome is complemented by thrombosis. In the tests, changes in the number of red blood cells, platelets and leukocyte shifts are observed. The counts of all blood cells may also increase, a phenomenon called “panmyelosis.”

The wasting stage is characterized by significant loss of body weight, asthenia and exhaustion.

For a newborn such clinical changes are extremely severe and can cause irreversible changes and subsequent death. Polycythemia can cause problems with the production of certain type leukocytes, which are responsible for immune system body. As a result, the baby develops severe bacterial infections, ultimately leading to death.

After reading this article, you learned more about such a pathology as polycythemia. We have reviewed the symptoms and treatment in as much detail as possible. We hope you find the information provided useful. Take care of yourself and be healthy!

Polycythemia vera (primary polycythemia) is an idiopathic chronic myeloproliferative disease, which is characterized by an increase in the number of red blood cells (erythrocytosis), an increase in hematocrit and blood viscosity, which can lead to the development of thrombosis. With this disease, hepatosplenomegaly may develop. In order to establish a diagnosis, it is necessary to determine the number of red blood cells and exclude other causes of erythrocytosis. Treatment consists of periodic bloodletting, and in some cases myelosuppressive drugs are used.

ICD-10 code

D45 Polycythemia vera

Epidemiology

Polycythemia vera (PV) is more common than other myeloproliferative diseases; the incidence is 5 cases per 1,000,000 people, men are more likely to get sick (ratio about 1.4:1). Middle age patients at the time of diagnosis are 60 years old (from 15 to 90 years old; this disease is rare in children); at the time of onset of the disease, 5% of patients are under 40 years of age.

Causes of polycythemia vera

Pathogenesis

Polycythemia vera is characterized by increased proliferation of all cell lineages, including erythrocyte, leukocyte and platelet lineages. An isolated increase in erythrocyte proliferation is termed “primary erythrocytosis.” In polycythemia vera, increased red blood cell production occurs independently of erythropoietin (EPO). Extramedullary hematopoiesis is observed in the spleen, liver and other sites with the potential for hematopoiesis. The life cycle of peripheral blood cells is shortened. In the later stages of the disease, approximately 25% of patients have reduced erythrocyte lifespan and inadequate hematopoiesis. Anemia, thrombocytopenia, and myelofibrosis may develop; precursors of erythrocytes and leukocytes can exit into systemic blood flow. Depending on the treatment, the frequency of transformation of the disease into acute leukemia varies from 1.5 to 10%.

With polycythemia vera, the volume and viscosity of the blood increases, which creates a predisposition to thrombosis. Because platelet function is impaired, the risk of bleeding is increased. A sharp intensification of metabolism is possible. Reduction life cycle cells leads to hyperuricemia.

Symptoms of polycythemia vera

Polycythemia vera is often asymptomatic. Sometimes increased blood volume and viscosity are accompanied by weakness, headaches, dizziness, visual disturbances, fatigue and shortness of breath. Itching is common, especially after a hot shower/bath. Facial hyperemia and congestion of retinal veins may be observed. Lower limbs may be hyperemic, hot to the touch and painful, and sometimes there is digital ischemia (erythromelalgia). An enlarged liver is characteristic; in addition, 75% of patients also have splenomegaly, which can be very pronounced.

Thrombosis can occur in various vessels, resulting in possible strokes, transient ischemic attacks, deep vein thrombosis, myocardial infarction, retinal artery or vein occlusions, splenic infarctions, or Budd-Chiari syndrome.

Bleeding (usually in the gastrointestinal tract) occurs in 10-20% of patients.

Complications and consequences

Diagnosis of polycythemia vera

IP should be excluded in patients with characteristic symptoms(especially in the presence of Budd-Chiari syndrome), however, the first suspicion of this disease often arises when abnormalities in the general blood test are detected (for example, with Ht > 54% in men and > 49% in women). The number of neutrophils and platelets may be increased, and the morphological structure of these cells may be disrupted. Since PV is a panmyelosis, the diagnosis is not in doubt in the case of proliferation of all 3 peripheral blood lineages in combination with splenomegaly in the absence of reasons for secondary erythrocytosis. However, all of these changes are not always present. In the presence of myelofibrosis, anemia and thrombocytopenia, as well as massive splenomegaly, may develop. In the peripheral blood, precursors of leukocytes and erythrocytes are found, pronounced anisocytosis and poikilocytosis are observed, microcytes, elliptocytes and drop-shaped cells are present. A bone marrow examination is usually performed and reveals panmyelosis, enlarged and aggregated megakaryocytes, and (sometimes) reticulin fibers. Cytogenetic analysis of the bone marrow sometimes reveals an abnormal clone characteristic of myeloproliferative syndrome.

Since Ht reflects the proportion of red blood cells per unit volume whole blood, an increase in Ht levels can also be caused by a decrease in plasma volume (relative or false erythrocytosis, which is also called stress polycythemia or Gaisbeck syndrome). As one of the first tests that helps distinguish polycythemia vera from increased hematocrit due to hypovolemia, it was proposed to determine the number of red blood cells. It should be taken into account that in polycythemia vera the plasma volume may also be increased, especially in the presence of splenomegaly, which makes Ht falsely normal despite the presence of erythrocytosis. Thus, for the diagnosis of true erythrocytosis, an increase in the erythrocyte mass is necessary. When determining the erythrocyte mass using erythrocytes labeled with radioactive chromium (51 Cr), the erythrocyte mass is more than 36 ml/kg in men (norm 28.3 ± 2.8 ml/kg) and more than 32 ml/kg in women (norm 25. 4 + 2.6 ml/kg) is considered pathological. Unfortunately, many laboratories do not perform blood volume tests.

Diagnostic criteria for polycythemia vera

Erythrocytosis, absence of secondary polycythemia and characteristic changes bone marrow (panmyelosis, enlarged megakaryocytes with aggregates) S combined with any of the following factors:

• Splenomegaly.
• Plasma erythropoietin level
• Platelet count > 400,000/µl.
• Positive endogenous colonies.
• Neutrophil count > 10,000/µl in the absence of infection.
• Clonal cytogenetic abnormalities in the bone marrow

It is necessary to think about the causes of erythrocytosis (of which there are quite a few). The most common secondary erythrocytosis due to hypoxia (HbO 2 concentration in arterial blood

The level of serum EPO in patients with polycythemia vera is usually reduced or normal, with erythrocytosis caused by hypoxia - increased, with tumor-associated erythrocytosis - normal or increased. Patients with increased level EPO or microhematuria should be evaluated with CT to look for renal pathology or other EPO-secreting tumors leading to secondary erythrocytosis. Unlike bone marrow healthy people, culture of bone marrow from patients with polycythemia vera can form red blood cell colonies without the addition of EPO (ie, positive endogenous colonies).

Although with polycythemia vera there may be various abnormalities in other laboratory tests, most of them are not necessary: ​​vitamin B12 levels and B12-binding capacity are often elevated, but these tests are not economically feasible. A bone marrow biopsy is also usually not necessary: ​​when performed, it usually reveals hyperplasia of all blood lines, accumulations of megakaryocytes, decreased iron stores (best assessed in bone marrow aspirate) and increased reticulin levels. Hyperuricemia and hyperuricosuria occur in more than 30% of patients. Recently, new diagnostic tests have been proposed: detection of increased expression of the PRV-1 gene in leukocytes and decreased expression of C-Mpl (receptor for thrombopoietin) on megakaryocytes and platelets.

Treatment of polycythemia vera

Since polycythemia vera is the only form of erythrocytosis for which myelosuppressive therapy can be indicated, it is very important to make an accurate diagnosis. Therapy should be carried out individually, taking into account the age, gender, general condition of the patient, clinical manifestations of the disease and hematological parameters.

Phlebotomy. Phlebotomy reduces the risk of thrombosis, improves symptoms and may be the only method of therapy. Phlebotomy is the treatment of choice in women childbearing age and patients under 40 years of age, as it does not have a mutagenic effect. Typically, the indication for phlebotomy is an Ht level greater than 45% in men and greater than 42% in women. At the beginning of therapy, 300-500 ml of blood is exfused every other day. A smaller volume of exfusions (200-300 ml twice a week) is performed in elderly patients, as well as patients with concomitant cardiac and cerebrovascular pathology. Once the hematocrit has been reduced below the threshold value, it should be determined once a month and maintained at this level with additional bloodletting (as needed). Before carrying out planned surgical interventions the red blood cell count should be reduced using phlebotomy. If necessary, intravascular volume can be maintained by infusions of crystal oid or colloid solutions.

Aspirin (at a dose of 81-100 mg orally 1 time per day) reduces the incidence of thrombotic complications. Patients undergoing phlebotomy alone or phlebotomy in combination with myelosuppressive therapy should take aspirin unless contraindicated.

Myelosuppressive therapy. Myelosuppressive therapy may be indicated for patients with platelet levels greater than 1/µl, with a feeling of discomfort due to increased visceral organs, with the presence of thrombosis, despite Ht less than 45%, symptoms of hypermetabolism or uncontrolled itching, as well as patients over 60 years of age or patients with cardiovascular diseases who do not tolerate bloodletting well.

Radioactive phosphorus (32 P) is effective in 80-90% of cases. The duration of remission ranges from 6 months to several years. P is well tolerated, and if the disease is stable, the number of follow-up visits to the clinic can be reduced. However, P therapy is associated with an increased rate of leukemic transformation, and when leukemia develops after phosphorus treatment, it is often resistant to induction chemotherapy. Thus, P therapy requires careful patient selection (eg, only used in patients likely to die from other disorders within 5 years).

Hydroxyurea is an inhibitor of the enzyme ribonucleoside diphosphate reductase - long time has been used for myelosuppression, its leukemic potential continues to be studied. Ht is reduced to less than 45% through phlebotomy, after which patients receive hydroxyurea at a dose of 20-30 mg/kg orally once daily. Patients are monitored weekly with a complete blood count. When a stable condition is achieved, the interval between control blood tests is extended to 2 weeks, and then to 4 weeks. When the level of leukocytes decreases to less than 4,000/μl or platelets to less than 100,000/μl, hydroxyurea is suspended; when the values ​​are normalized, it is resumed at a dose reduced by 50%. In patients with unsatisfactory disease control, requiring frequent phlebotomies, or patients with thrombocytosis (platelet level > 600,000/μl), the dose of the drug may be increased by 5 mg/kg monthly. Acute toxicity is rare, and rash, GI symptoms, fever, nail changes, and skin ulceration may occasionally occur and may require discontinuation of hydroxyurea.

Interferon a2b was used in cases where blood cell levels could not be controlled with hydroxyurea or when the drug was poorly tolerated. The usual starting dose is 3 units subcutaneously 3 times a week.

Anagrelide is a new drug that has a more specific effect on megakaryocyte proliferation than other drugs and is used to reduce platelet levels in patients with myeloproliferative diseases. The safety of this drug during long-term use is currently being studied, but according to available data, it does not contribute to the progression of the disease to acute leukemia. When using the drug, vasodilation may develop with headaches, palpitations and fluid retention. To minimize the indicated side effects The drug is started at an initial dose of 0.5 mg twice daily, then the dose is increased weekly by 0.5 mg until the platelet count decreases to less than 450,000/mcL or until the dose is 5 mg twice daily. The average dose of the drug is 2 mg/day.

Most alkylating agents and, to a lesser extent, radiophosphorus (formerly used for myelosuppression) have leukemoid effects and should be avoided.

Treatment of complications of polycythemia vera

For hyperuricemia, if it is accompanied by symptoms or if the patient is simultaneously receiving myelosuppressive therapy, it is necessary to take allopurinol 300 mg orally once a day. Itching may be relieved after taking antihistamines, however, this does not always happen; The most effective treatment for this complication is often myelosuppressive therapy. To relieve itching, cholestyramine 4 g orally three times a day, cyproheptadine 4 mg orally 3-4 times a day, cimetidine 300 mg orally 4 times a day, paroxetine 20-40 mg orally once a day can also be used. After the bath, the skin must be dried carefully. Aspirin relieves symptoms of erythromelalgia. Elective surgical interventions for polycythemia vera should be performed only after the Ht level has decreased

A blood disease accompanied by an increased volume of red blood cell lineage cells is called polycythemia. Depending on the causes, pathology is divided into true (primary) and secondary.

The studied prevalence of polycythemia vera allows us to identify the most dangerous group of middle-aged and elderly people, most often men. Every year, 4–5 primary cases are registered for every million of the population.

In newborns, polycythemia may occur secondarily in response to oxygen hypoxia of tissues.

Types of disease

The types of disease differ in the severity of the course and the main connection with the mechanism of damage to the hematopoietic organs.

• Polycythemia vera is always a manifestation of tumor-like proliferation of precursor cells normal red blood cells.
• Secondary polycythemia is formed under the influence of various diseases that cause “thickening” of the blood.

These could be:

• dehydration factor (loss of fluid from frequent and profuse vomiting due to cholera, poisoning, diarrhea, large burn surface);
• hypoxia (lack of oxygen) caused by climbing mountains, heat, and excessive sweating during fever.

The secondary state is clinical manifestation another disease, such as salmonellosis or dysentery. At the same time, the total mass of red blood cells in the blood of patients remains normal.

In hot climates, a person loses a lot of fluid through sweat; without drinking, polycythemia will occur.

A decrease in the plasma part leads to a relative shift towards high viscosity. Treatment of this pathology is always associated with compensation of fluid in the body. This leads to full restoration ratio of red blood cells and plasma.

Mechanisms of development of polycythemia

Dehydration and oxygen deficiency in tissues force the human body to compensate for the production of hemoglobin through additional synthesis of red blood cells (cells that carry oxygen molecules). At the same time, red blood cells produced by the bone marrow have the correct shape, volume, and have all the functions.

In contrast to this process, polycythemia vera is accompanied by a mutation of stem cells in the red bone marrow. The synthesized cells belong to the precursors of red blood cells, do not correspond to size, and are not needed by the body in large quantities.

Proven connection tumor growth with two cell populations:

• the first develops independently (autonomously) from defective progenitors thanks to genetic mutation;
• the second depends on the effect of erythropoietin, a hormone produced by the kidneys, which controls not only the production of the number of red blood cells, but also their correct step-by-step differentiation.

“Triggering” the mechanism of activation of erythropoietin explains the addition of secondary polycythemia to tumor process.

A huge number of unnecessary blood cells leads to thickening of the blood and provokes increased thrombus formation. Accumulation in the spleen, which does not have time to destroy the formed elements, leads to an increase in its mass and stretching of the capsule.

Main causes and risk factors

The main reason Primary polycythemia is considered to be a genetic inherited mutation. Genes have been found that are responsible for the synthesis of red blood cells and exhibit particular sensitivity to erythropoietin. This pathology is considered familial because it is found in relatives.
One of the variants of genetic changes is the pathology of genes, when they begin to capture more oxygen molecules, but do not release it to the tissues.

Polycythemia develops secondary to prolonged chronic diseases stimulating increased levels of erythropoietin. These include:

• chronic diseases respiratory organs with impaired patency ( obstructive bronchitis, asthma, emphysema);
• increased pressure in the pulmonary artery due to heart defects, thromboembolism;
• heart failure as a result of ischemia, decompensation of defects, consequences of arrhythmia;
• renal ischemia with atherosclerosis of afferent vessels, cystic tissue degeneration.

Varieties found in cancer diseases malignant tumors, activating the production of erythropoietin:

• liver carcinoma;
• renal cell carcinoma;
• uterine tumor;
• neoplasm of the adrenal glands.

Polycythemia in smokers occurs when oxygen in the inhaled air is replaced by carbon oxides and other toxic substances.

Risk factors may include:

• stressful situations;
• long-term living in high mountains;
• professional contact with carbon monoxide when working without protective masks in a garage, coal mines, electroplating shops.

Clinical manifestations

Symptoms of polycythemia appear in the second stage of the disease. Initial signs can only be detected through laboratory examination. They are often hidden behind the underlying disease.

• Patients complain of frequent headaches, dizziness, and a feeling of “heaviness” in the head.
• Itching of the skin begins gradually and becomes painful. He explains himself increased emissions mast cells histamine-like substances. Itching typically intensifies during a shower or bath, or when washing.
• Change in skin color - patients have a red, swollen face, their hands become purple with a bluish tint.
• Painful sensations in the fingers occur when touching objects.
• Characterized by a significant increase in systolic blood pressure(up to 200 mm Hg and above).
• Due to the enlargement of the spleen, pain appears in the left hypochondrium. The liver also reacts; upon examination, a protruding edge is detected.
• Pain in the bones (hips, along the ribs).
• Increased fatigue, tendency to acute infections.
• In the final stage, signs of bleeding appear: bruises on the body, bleeding from the nose, gums.

One of the symptoms is facial redness and injected sclera

The disease can be detected by detecting increased thrombus formation and clinical manifestations of stroke, acute infarction, manifestations of embolism in the mesenteric vessels (abdominal pain).

Stages of flow

The true form is characterized by 3 stages of the disease:

• the onset of the disease or its height - by clinical signs only changed ones can be detected laboratory parameters(erythrocytosis, increased hemoglobin and hematocrit), the patient has no complaints;
• second - all the symptoms and characteristic signs of hematopoietic disorders appear;
• third or final - anemia occurs due to bone marrow depletion, internal and external bleeding is added to the symptoms of the second period, sharp increase spleen and liver, manifestations of hemorrhage in the brain.

Treatment of polycythemia is based on a syndromic approach, depending on the identified period of the disease.

Development of pathology in children

IN childhood Polycythemia is most often found in newborns (neonatal form). It is detected in the first 2 weeks of a baby’s life. Its cause is the child’s body’s response to oxygen deficiency in the womb due to impaired placental nutrition.

Twins are especially susceptible to genetic changes. Judging by the blueness of the baby's skin, heart defects and respiratory disorders, which are accompanied by an increased level of red blood cells, are suspected. Hemoglobin increases 20 times.

The stages of the disease in newborns are the same as in adults. Due to pain and itching, the baby does not allow him to touch the skin. In children, other blood cells suffer much more quickly: thrombocytosis and leukocytosis appear.

The baby is not gaining weight, and exhaustion worsens the general condition. In the final stage, the production of immune cells stops. A child can die from any infection.

Diagnostics

The diagnosis of the disease is based on laboratory research blood:

1. When counting the number of red blood cells, their number is found to be from 6.5 to 7.5 x 10 12 per liter. An indicator of polycythemia is the excess of the total weight in men 36, in women 32 ml/kg of weight.
2. At the same time, there is leukocytosis and thrombocytosis in the first stages.
3. The laboratory technician sees in the smear large number red blood cell precursors (metamyelocytes).
4. From biochemical tests, attention is paid to the increase in alkaline phosphatase activity.

Modern equipment allows for quick and objective diagnosis

Bone marrow analysis allows a definitive diagnosis.

Treatment

It is important to consider the underlying cause of polycythemia in your treatment regimen. If secondary changes can be compensated and limited, then therapy for primary tumor cell proliferation is very difficult.

Restriction of the regimen is not required if thrombotic complications do not occur.

The diet must include a significant amount of fluid and limit foods that enhance hemoglobin synthesis and contain a lot of iron. These include: chicken, beef, turkey, liver in any form, fish, cereals - buckwheat and millet, chicken eggs. Fat broths are not shown. Dairy products are recommended.

Apply medicines, suppressing bone marrow activity (Hydroxycarbamide, Hydroxyurea). Cytostatics include Myelosan, Myelobromol.

Bloodletting allows you to slow down the pathological effect of the mass of red blood cells for a short period

It is acceptable to reduce the hematocrit during blood withdrawal to 46%. Before the first procedure, blood clotting indicators are studied, and Aspirin and Curantil are prescribed to prevent the risk of platelet aggregation. Drip administration of Reopoliglucin and Heparin is possible.

The volume of one-time intake is up to 500 ml (with concomitant heart failure - 300). The procedures are carried out in a course every other day.

Cytopheresis - blood purification using special filters. Allows you to retain some red blood cells and return your own plasma to the patient.

Traditional therapy

Treatment of polycythemia vera with folk remedies is very problematic, since such a method has not yet been found. Therefore, doctors recommend that patients refrain from using any advice from healers.

The most popular among them are decoctions of cranberries and sweet clover herbs. A medicinal tea is prepared from them and drunk throughout the day.

Forecast

The prognosis for the primary form of the disease is very unfavorable: without treatment, patients live for two years, no more. Death occurs from thrombosis or bleeding with brain damage.

Bloodletting and others modern methods therapy made it possible to prolong the life of patients by 15 years or more.

If changes are unexpectedly detected in blood tests, do not immediately become alarmed. An additional examination will, first of all, eliminate the possibility of improper preparation for the analysis (after stress, a night shift, or eating). This can significantly affect the results. If the diagnosis is confirmed, only rapid initiation of treatment will provide the necessary help.