Primary polycythemia. Polycythemia - stages and symptoms of the disease, hardware diagnostics and methods of therapy

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

Features of the disease

Primary (true) polycythemia is a blood disease from the leukemia group that occurs idiopathically (for no apparent reason), 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 Wakez-Osler disease, erythremia, primary erythrocytosis. The consequences of erythrocytosis and blood clotting in this myeloproliferative disease can be serious and relate to the risk of thrombosis, enlargement and malfunction of the spleen, an increase in circulating blood volume, etc.

Erythremia is considered a malignant tumor process, which is caused by increased proliferation (hyperplasia) of bone marrow cells. Particularly strongly pathological process covers erythroblastic germ - part of the bone marrow, consisting of erythroblasts and normoblasts. The pathogenesis of the main manifestations is associated with the appearance in the blood of a huge number of red blood cells, as well as with a certain increase in the number of platelets and neutrophils (neutrophilic leukocytes). The 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 blood flow, the formation of blood clots, a violation of the local blood supply to tissues and their hypoxia.

If initially the patient most often has primary erythrocytosis, that is, only the number of erythrocytes increases, then further changes begin to cover other blood cells. Extramedullary hematopoiesis (pathological formation of blood outside the bone marrow) occurs in the organs of the peritoneum - in the liver and spleen, where part of erythropoiesis is also localized - the process of formation of red blood cells. 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 circulation without maturing. In about 10% of cases, the pathology flows into acute leukemia.

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

Causes of pathology

The primary form of the disease is considered hereditary, transmitted in an autosomal recessive manner. In this case, it is often referred to as "family 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 in many patients the clonal origin of the pathology, since the same enzyme was detected in leukocytes, platelets, erythrocytes. The clonal theory is also confirmed by ongoing cytological studies in relation to the karyotype of chromosome groups, where various defects were identified that are similar in different patients. There is also a virus-genetic theory, according to which up to 15 types of viruses can enter the body and, with the participation of a number of provoking factors, lead to a malfunction in the bone marrow. They penetrate into the precursors of blood cells, which then, instead of normal maturation, begin to divide and form new red blood cells and other cells.

As for the risk factors for the development of true polycythemia, then, presumably, they may be as follows:

• lung diseases;
• long stay at high altitude above sea level;
• hypoventilation syndromes;
• various hemoglobinopathies;
• long smoking history;
• tumors of the 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 with vapors of chemicals, their ingress through the skin;
• the intake of toxic substances in the digestive tract;
• treatment with gold salts;
• advanced tuberculosis;
• major surgical interventions;
• "blue" heart defects;
• kidney pathology - hydronephrosis, stenosis of the arteries of the kidneys.

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

Classification of true polycythemia

The disease is classified into the following stages:

1. The first or initial stage. It can last more than 5 years, is the development of plethoric syndrome, that is, increased blood supply to organs. At this stage, the symptoms can be expressed moderately, complications do not occur. A complete blood count reflects a slight increase in the number of erythrocytes, a bone marrow puncture shows an increase in erythropoiesis or the production of all the main blood elements, with the exception of lymphocytes.
2. The second is A stage, or polycythemic stage. Duration - from 5 to 15 years. The plethoric syndrome is more pronounced, there is an increase in the spleen, liver (hematopoietic organs), thrombosis is often recorded in the veins and arteries. Tumor growth in the organs of the peritoneum is not observed. 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 erythrocytes, platelets, leukocytes, with a running course - a decrease in platelets. In the myelogram, there is an increased formation of most blood cells (with the exception of lymphocytes), cicatricial changes in the brain are formed.
3. The second - stage B, or polycythemic stage with myeloid metaplasia of the organ - the spleen. The patient continues to increase the size of the spleen, and often the liver. Puncture of the spleen reveals tumor growth. There are frequent thromboses interspersed with bleeding. In the general analysis, there is an even greater increase in the number of erythrocytes, leukocytes, there are erythrocytes of different sizes, shapes, there are immature precursors of all blood cells. In the bone marrow, the number of cicatricial changes increases.
4. The third, or anemic stage. It is the outcome of a disease in which the activity of blood cells is depleted. The number of erythrocytes, leukocytes, and platelets is greatly reduced, the liver and spleen are enlarged with myeloid metaplasia, extensive cicatricial changes occur 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 recorded 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 disease in a patient manifests itself in one way or another, while specific symptoms can be varied. Basically, the symptom complex includes the following main signs:

1. Change in skin tone, dilated veins. Most often, in the neck of an adult, the veins begin to shine through strongly, their pattern becomes stronger due to swelling, overfilling with blood. But the skin signs become the most obvious: the skin color becomes dark red, literally cherry. Most of all, this is noticeable in the neck, arms, face, which is associated with overfilling of the subcutaneous arteries with blood. At the same time, many patients mistakenly think that blood pressure rises against the background of hypertension, and therefore they often continue to take drugs for pressure and do not go to the doctor. With a careful attitude to health, you can see that the lips and tongue also changed their shade, became red-blue. The vessels of the eyes are also filled with blood, their plethora leads to hyperemia of the sclera and conjunctiva of the organs of vision. The hard palate remains the same color, but the soft palate also becomes brighter, burgundy.
2. Skin itching. All described changes in the skin in about half of the cases are complemented by severe discomfort and itching. This symptom is very characteristic of erythremia, both primary and secondary. Since after taking water procedures in patients, histamine is released, as well as prostaglandins, itching of the skin may become even more pronounced after a bath or shower.
3. Pain in limbs. Many people develop obliterating endarteritis, which results in persistent and severe pain in the legs. They can intensify with exercise, long walking, in the evening, at first they are often perceived as a symptom of fatigue in an elderly person. Pain is also observed during palpation and tapping of flat bones, which reflects the process of hyperplasia and scarring in the bone marrow. The next type of pain in a person with polycythemia vera is persistent burning pain in the large and small joints of the legs, which resemble gouty pains and are caused by the same cause 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, blue spots appear on it. These pains are caused by an increase in the number of platelets and the appearance of microthrombosis of capillaries.
4. Splenomegaly. The growth of the spleen in size is observed in almost every person with polycythemia vera, but at different stages of the disease. This is due to increased filling of the spleen with blood and the development of myeloproliferative phenomena. Somewhat less often, but still there is a strong increase in the size of the liver - hepatomegaly.
5. Ulcer disease. Approximately one in ten people with Wackez-Osler disease develop ulcers in the small intestine (often in the duodenum) and in the stomach. This is due to the activation of Helicobacter pylori bacteria, as well as the development of microthrombosis in the gastrointestinal tract.
6. thrombosis and bleeding. Almost all patients at a certain stage have a tendency to thrombosis, and until recently, patients died from such complications at an early stage of the disease. Now ongoing modern treatment can prevent the appearance of blood clots in the brain, spleen, legs, which threaten embolism and death. Increased blood viscosity characterizes true polycythemia in the initial stages, and later, against the background of the 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 specific and may be inherent in various pathologies:

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

In general, the disease is characterized by a long and sometimes benign course, especially when adequate treatment is carried out. But in some people, especially those who are not receiving therapy, various consequences of polycythemia vera may appear early.

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 thrombus, the affected area. Transient ischemic attacks, strokes, thrombophlebitis and phlebothrombosis of superficial and deep veins, blockage of retinal blood vessels and blindness, heart attacks of internal organs, myocardial infarction may occur.

In the most advanced stages of the pathology, kidney stones (urolithiasis), gout, nephrosclerosis, and cirrhosis of the liver often appear. The occurrence of complications due to tissue bleeding is likely - bleeding from gastrointestinal ulcers, anemia. From the side of the heart, in addition to myocardial infarction, signs of myocardiosclerosis and heart failure are also possible. There is also the possibility of the transition of true polycythemia to acute leukemia, chronic leukemia and other oncopathologies.

Carrying out diagnostics

It is not easy to make a diagnosis of this disease, especially in the absence of a characteristic clinical picture and in the presence of only general symptoms. However, the combination of data from hematological and biochemical analyzes, 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.

The main indicators in order to establish the diagnosis of polycythemia vera are the indicators of the general blood test - the number of erythrocytes 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, hematocrit was above 52%. In women, an excess of indicators is 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 pathology, and as it develops, they become even higher. In addition, it is important to assess the mass of circulating erythrocytes, which is normally up to 36 ml/kg for men and up to 32 ml/kg for women.

Other blood parameters (according to 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 of an increased number of basophils and eosinophils.
2. Increase in the number of reticulocytes.
3. The appearance in the blood of myelocytes, metamyelocytes.
4. Increase in blood viscosity by 500-800%.
5. A strong decrease in ESR.
6. Increase in the mass of circulating red blood cells.
7. Increase in alkaline phosphatase, vitamin B12 in serum.
8. An increase in the amount of uric acid in the serum.
9. The saturation of blood in the arteries with oxygen is above 92%.
10. The appearance of erythrocyte colonies in a test tube.
11. Decrease in erythropoietin.
12. Change in color index less than 1.

At the stage of myelofibrosis, hemoglobin and erythrocyte indicators can return to normal, but 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 in it:

• reduction in 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 ongoing changes characteristic of polycythemia vera:

1. Hepatosplenomegaly.
2. Tendency to thrombosis.
3. Increased sweating combined with weight loss, weakness.
4. The presence of gene abnormalities, if genetic testing has been performed, when it comes to primary erythremia.
5. Increase in the average amount of circulating blood.

All the criteria described above, except for the three main ones, which are considered large, are small. As for the major diagnostic criteria, this is an increase in the mass of circulating erythrocytes, splenomegaly, supersaturation of arterial blood with oxygen. To establish 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 earlier a person seeks help, the more effective the therapy can be. At the third stage, or when layering another tumor process on erythremia, symptomatic therapy is performed in combination with chemotherapy treatment. Chemotherapeutic treatment may be recommended at other stages of the disease, but the body does not always give an adequate response to it. Of the symptomatic means that improve the quality of life, the following are used:

1. Drugs against high blood pressure, 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 bleeding tissues.
5. Medications to lower uric acid.

Treatment options for polycythemia vera may include:

1. Bloodletting, or removing a small amount of blood from the bloodstream (phlebotomy). As a rule, they are made in a volume of 100-400 ml (according to indications) and a break of 3-4 days in a course of several sessions. The blood after such manipulations becomes more liquid, but they cannot be done if there is a recent history of blood clots. Before treatment with bloodletting, the patient is given a solution of Reopoliglyukin, as well as Heparin.
2. Erythrocytapheresis. It is used to clean the blood from excess red blood cells, as well as from platelets. Such sessions are done once a week.
3. Chemotherapy. It is used, as a rule, when the disease reaches the tumor stage - the second B. Other indications for chemotherapy are the presence of complications from the peritoneal organs, the general plight of a person, and an increase in the number of all blood elements. For chemotherapy, or cytoreductive therapy, cytostatics, antimetabolites, alkylating drugs, biological drugs are used. The most commonly prescribed drugs are Leukeran, Hydroxyurea, Mielosan, 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 spleen area and stop the cancer process in it, it is used with a strong increase in the size of the organ.
6. Transfusion of erythrocyte mass from purified erythrocytes. Used for severe anemia up to coma. If thrombocytopenia increases in the final stages of polycythemia vera, a platelet mass transfusion from a donor may be necessary.

Bone marrow transplantation for a disease such as erythremia often leads to adverse results, so it is 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 of gestation. However, in 50% of cases, pregnancy ends in a successful birth. The remaining half is accounted for by miscarriages, developmental delay, anomalies in the structure of the fetal body.

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, bloodletting therapy and, if necessary, glucocorticosteroids are mainly performed. To prevent complications and early detection of the disease in pregnant women, blood tests should be carried out regularly according to the schedule indicated by the observing obstetrician-gynecologist.

What Not to Do

It is strictly forbidden to use diuretics, which additionally thicken the blood. Also in our time, the use of radioactive phosphorus preparations is limited, which seriously inhibit myelopoiesis and often lead to the development of leukemia. Also, you can not save the old nutrition system: the diet must change. All foods that enhance blood formation, such as the liver, are banned. It is better to form a diet as a dairy-vegetable one, and refuse excess meat.

The patient should not overload the body, engage in heavy sports, ignore regular rest. Treatment with folk remedies can be used, but only after a thorough study of all the remedies by the doctor in terms of 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

Methods of prevention have not yet been developed. The prognosis for life varies depending on the severity of the course of the disease. Without treatment, up to a third of patients die within the first 5 years of diagnosis. If you carry out a full-fledged therapy, then you can extend the life of a person up 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 heavy bleeding.

Today we will talk about such a blood disease as polycythemia vera. This disease is a pathology in which there is an increased number of red blood cells in the circulating blood. Polycythemia carries a great, sometimes irreversible danger to human life and health, so it is important to recognize the disease by its first signs for timely medical care and competent treatment. Typically, this syndrome is characteristic of people over the age of 50, and is more often diagnosed in males. Let us consider in more detail the disease in all its aspects: etiology, types, diagnosis and main methods of curing polycythemia.

General information about the disease

In modern medicine, polycythemia has several names, for example, Wakez's disease, it is also sometimes called erythrocytosis. Pathology belongs to the section of chronic leukemia and represents an active increase in the concentration of erythrocytes, leukocytes and platelets in the blood, most often specialists refer this disease to a rare type of leukemia. Medical statistics say that true polycythemia is diagnosed annually only in 5 cases per 1 million patients, usually the development of pathology is typical for older men (from 50 to 65 years).

The most dangerous complications of the disease include the risk of developing thrombosis and hemorrhagic strokes, as well as the transition of polycythemia to the acute stage of myeloid leukemia or to the chronic stage of myeloid leukemia. This disease is characterized by a number of reasons, which we will consider below. All causes of erythremia are divided into two types: primary and secondary.

Causes of the disease

In modern medicine, the root causes of this pathology include the following:

• genetic predisposition to increased production of red blood cells;
• failures at the genetic level;
• oncological diseases of the bone marrow;
• oxygen deprivation also affects the increased production of blood cells.

Most often, erythremia has a tumor factor, characterized by damage to stem cells produced in the red bone marrow. The result of the destruction of these cells is an increase in the level of erythrocytes, which directly leads to disruption of the whole organism. The disease is malignant, difficult to diagnose and treated for a long time, and not always with a positive effect, complex therapy is due to the fact that no treatment methods can affect a stem cell that has undergone a mutation, which has a high ability to divide. True polycythemia is characterized by the presence of plethora, this is due to the fact that the concentration of red blood cells is increased in the vascular bed.

In patients with polycythemia, purplish-red skin is observed, often patients complain of itching.

The secondary causes of the disease experts include factors such as:

• pathology of the lungs of an obstructive nature;
• pulmonary hypertension;
• heart failure in a chronic form;
• there is not enough oxygen supply to the kidneys;
• a sharp change in climate, and the development of this syndrome is typical for the population living in high mountainous areas;
• various infections leading to high intoxication of the body;
• harmful working conditions, especially for work carried out at height;
• the disease also affects people living in ecologically polluted areas, or in close proximity to industries;
• excessive smoking;
• experts have revealed that a high risk of developing polycythemia is characteristic of people with Jewish roots, this is due to the genetic feature of the function of the red bone marrow;
• sleep apnea;
• hypoventilation syndromes leading to polycythemia.

All these factors lead to the fact that hemoglobin is endowed with the ability to actively attach oxygen to itself, while there is practically no return to the tissues of internal organs, which, accordingly, leads to the active production of red blood cells.

It is worth noting that some cancers can also provoke the development of erythremia, for example, tumors of the following organs affect the production of red blood cells:

• liver;
• kidneys;
• adrenal glands;
• uterus.

Some kidney cysts and obstruction of this organ can increase the secretion of blood cells, leading to the development of polycythemia. Sometimes polycythemia occurs in newborns, this disease is transmitted through the maternal placenta, there is an insufficient supply of oxygen to the fetus, as a result of which pathology develops. Next, consider the course of polycythemia, its symptoms and treatment, what are the complications of polycythemia?

Symptoms of polycythemia

This disease is dangerous because polycythemia vera at the initial stage is almost asymptomatic, the patient does not have any complaints about deteriorating health. Most often, pathology is detected during a blood test, sometimes the first “rings” of polycythemia are associated with colds or simply with a general decrease in efficiency in the elderly.

The main signs of erythrocytosis include:

• a sharp drop in visual acuity;
• frequent migraines;
• dizziness;
• noise in ears;
• sleep problems;
• "icy" fingers.

When the pathology enters the advanced stage, then with polycythemia, the following can be observed:

• muscle and bone pain;
• ultrasound is often diagnosed with an enlarged spleen, or a change in the contours of the liver;
• bleeding gums;
• for example, when a tooth is removed, the blood may not stop for a long time;
• patients often find new bruises on their bodies, the origin of which they cannot explain.

Doctors also identify specific symptoms of this disease:

• severe skin itching, which increases after taking water procedures;
• burning sensation of the fingertips;
• the appearance of vascular "asterisks";
• the skin of the face, neck and chest may acquire a purple-red hue;
• lips and tongue, on the contrary, may have a bluish tint;
• the whites of the eyes are prone to redness;
• the patient constantly feels weak.

If we talk about a disease that affects newborns, then polycythemia develops a few days after birth. Most often, pathology is diagnosed in twins, the main signs include:

• the baby's skin turns red;
• when touching the skin, the child experiences discomfort, so he starts to cry;
• the baby is born small;
• a blood test reveals an increased level of leukocytes, platelets and erythrocytes;
• on ultrasound, changes in the size of the liver and spleen are observed.

It should be noted that if polycythemia is not diagnosed in time, the development of the disease can be missed, and the lack of therapy can lead to the death of the newborn.

Diagnosis of the disease

As mentioned above, most often true polycythemia is detected during prophylactic blood donation for analysis. Specialists diagnose erythrocytosis if, in a blood test, the indicators show a level above the norm:

• hemoglobin level increased to 240 g/l;
• the level of erythrocytes is increased to 7.5x10 12 /l;
• the level of leukocytes is increased to 12x10 9 /l;
• platelet level increased to 400x10 9 /l.

To study the function of the red bone marrow, a trepanobiopsy procedure is used, because it is the violation of stem cell production that provokes the development of polycythemia. To exclude other diseases, specialists can use studies such as ultrasound, urinalysis, FGDS, ultrasound, and so on. Also, the patient is prescribed consultations with narrow specialists: a neurologist, a cardiologist, a urologist, etc. If a patient is diagnosed with polycythemia, what is the treatment for this disease, consider the main methods.

Treatment of erythrocytosis

This disease belongs to those types of pathology that are treated with myelosuppressive drugs. Also, the treatment of polycythemia vera is carried out by methods of bloodletting, this type of therapy can be prescribed to patients who have not reached the age of 45. The essence of the procedure is that up to 500 ml of blood is taken from the patient per day, phlebotomy is also performed for elderly people with polycythemia, only blood is taken no more than 250 ml per day.

If a patient with this disease has severe itching and hypermetabolic syndrome, then specialists prescribe a myelosuppressive method of treating polycythemia vera. It includes the following drugs:

• radioactive phosphorus;
• anagrelide;
• interferon;
• hydroxyurea.

In the case of remission with polycythemia, the patient is prescribed repeated blood tests no more than once every 14 days, then the study is carried out once a month. When the level of erythrocytes returns to normal, the drugs begin to be gradually canceled, there is an alternation of drug therapy with rest from drugs, while the course of the disease is strictly observed. But it is worth noting that the use of myelosuppressive drugs in polycythemia can lead to the development of leukemia, so specialists prescribe them after lengthy detailed studies. Sometimes there are side effects such as skin ulcers, disruption of the gastrointestinal tract, fever, if this happens, then the medications are immediately canceled.

Also, the patient should take Aspirin daily to reduce the risk of developing thrombosis, which often complicates the course of this disease.

A patient with polycythemia is also shown a procedure such as erythrocytophoresis, which consists in the fact that the device pumps out blood from the patient, while simultaneously removing excess red blood cells from it. After that, in order to restore the previous volume, the patient is infused with saline, this procedure is a modern type of bloodletting, but it is carried out no more than once every 2-3 years. Treatment of polycythemia does not insure the patient against possible complications that may develop against the background of this pathology.

Complications of polycythemia

Experts note the following complications that accompany the development of true polycythemia:

• urine can acquire a sharp and unpleasant odor;
• often patients with polycythemia suffer from gout;
• with polycythemia, kidney stones can form;
• renal colic pass into the chronic stage;
• often erythrocytosis accompanies a stomach or duodenal ulcer;
• impaired circulatory function can lead to the formation of skin ulcers;
• often this disease provokes thrombosis;
• bleeding gums, frequent nosebleeds.

Preventive measures

The development of a disease such as polycythemia can be prevented, it is necessary to adhere to the following preventive measures:

• completely abandon bad habits, especially cigarette smoking, it is nicotine that harms the body and provokes this disease;
• if the area is unfavorable for living, then it is better to change the place of residence;
• the same applies to work;
• regularly take preventive blood tests, which can show the presence of polycythemia in the patient;
• it is necessary to take a responsible attitude to your diet, it is better to limit the consumption of meat, include in your diet those foods that stimulate the function of hematopoiesis, give preference to sour-milk and vegetable products.

Remember that timely diagnosis and competent treatment of polycythemia can prevent the development of complications in this disease, but, unfortunately, with this disease there is no guarantee of a complete cure.

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Erythremia (polycythemia vera, Wakez's disease) is a hereditary disease of the blood system, occurring mainly in elderly women.

An increase in the number of red blood cells in polycythemia vera

This pathology is characterized by malignant hypertrophy of the bone marrow. Most often, this pathology is known to patients as blood cancer (although such a judgment is erroneous) and leads to a progressive increase in the number of blood cells, primarily red blood cells (the number of other elements also increases). As a result of an increase in their number, an increase in hematocrit is observed, which leads to a decrease in the rheological properties of the blood, a decrease in the rate of blood flow through the vessels, and as a result, an increase in thrombus formation and deterioration in tissue supply.

These reasons lead to the fact that most tissues experience oxygen starvation, which reduces their functional activity (ischemic syndrome). Polycythemia vera occurs mainly in women. Men get sick a little less often, the frequency of occurrence of this pathology is approximately 3:2.

On average, Wakez disease occurs around the age of 40, and the peak of symptoms occurs in the 60s and 70s. There is a hereditary predisposition to the disease. In the population, erythremia is quite rare - about 30 cases per million population.

The main symptoms of the disease

Erythremia is an excessive saturation of the blood with red blood cells, which leads to various tissue and vascular disorders. Among the most common symptoms are:

1. Change in skin color. The main causes are stagnation of blood and restoration of hemoglobin. Due to reduced blood flow, red blood cells stay in one place longer, which leads to the restoration of the hemoglobin contained in them, and as a result, a change in the color of the skin. Patients suffering from this disease have a characteristic appearance - a reddened face and an intensely cherry-colored neck. In addition, swollen veins are clearly visible under the skin. When studying the mucous membranes, one can observe the characteristic symptom of Kuperman - a change in the color of the soft palate with an unchanged color of the hard palate.
2. Itching. This syndrome develops due to an increase in the number of immune cells that have the ability to release specific inflammatory mediators, in particular, serotonin and histamine. Itching intensifies after mechanical contact (most often after a shower or bath).
3. Erythromelalgia - discoloration of the distal phalanges of the fingers with the appearance of pain. This syndrome is caused by an increased content of platelets in the blood, which leads to clogging of small capillaries of the distal phalanges, the development of an ischemic process and pain in their tissues.
4. Splenitis and hepatomegaly. An increase in these organs is observed in most hematological diseases. If a patient develops erythremia, then an increased concentration of cells in the blood can lead to an increase in blood flow in these organs, and as a result, their increase. This can be determined by palpation or instrumental studies. The megalia syndrome is eliminated on its own after the normalization of the hemogram, that is, when the blood test returns to normal.
5. Thrombosis. Due to the high concentration of cells in the blood and a decrease in blood flow, a large number of blood clots form in the areas of damage to the intima of blood vessels, which lead to blockage of blood vessels in all parts of the body. The development of thrombosis of mesenteric, pulmonary or cerebral vessels is especially dangerous. In addition, blood clots in the small vessels of the gastric mucosa lead to a decrease in its protective properties and the appearance of gastritis and ulcers. DIC syndrome may also occur.
6. Pain. It can develop as a result of vascular disorders, for example, with obliterating endarteritis, and as a result of some metabolic disorders. With polycythemia, there may be an increase in the level of uric acid in the blood, its deposition in the joints. In rare cases, pain is observed during percussion or tapping of flat bones containing bone marrow (due to its hyperplasia and stretching of the periosteum).

Among the general symptoms, if erythremia occurs, headache, dizziness, a feeling of heaviness in the head, tinnitus, general weakness syndrome (all symptoms are due to a decrease in tissue oxygenation, impaired blood circulation in certain parts of the body) come first. When diagnosing, they are not used as mandatory criteria, since they can correspond to any systemic disease.

Stages and degrees of polycythemia

True polycythemia occurs in three stages (stages):

• stage of initial manifestations. At this stage, the patient does not present specific complaints. He is concerned about general weakness, increased fatigue, a feeling of discomfort in the head. All these symptoms are most often attributed to overwork, social and life problems, which is why the disease itself is diagnosed quite late;
• extended stage (clinical stage). At this stage, the appearance of a headache, discoloration of the skin and mucous membranes is characteristic. The pain syndrome develops quite late and indicates the neglect of the disease;
• terminal stage. At this stage, damage to internal organs due to their ischemia, dysfunction of all body systems are most pronounced. There may be a lethal outcome due to secondary pathology.

All stages proceed sequentially, and the diagnosis of the disease (blood test) becomes informative from the stage of clinical signs.

Diagnosis of Wakez disease

To make a diagnosis, a complete blood count plays a decisive role. It shows pronounced erythrocytosis, an increase in hemoglobin and hematocrit. The most reliable is the analysis of bone marrow punctate, which reveals signs of hyperplasia of the erythroid germ, and also calculates how many cells are present in it and what is their morphological distribution.

To clarify the nature of concomitant pathology, it is recommended to conduct a biochemical analysis, which provides information about the state of the liver and kidneys. With massive thrombosis, the state of blood coagulation factors is assessed by analyzing it for coagulability - a coagulogram.

Other studies (ultrasound, CT, MRI) give only an indirect idea of ​​the state of the body and are not used in making a diagnosis.

Treatment of erythremia

Despite the variety and severity of the manifestations of Wakez disease, there are relatively few treatments for it. It depends on what the analysis of the hemogram showed, whether a cytological syndrome has developed and what symptoms the patient has.

As mentioned above, the cause of the disease is an increased concentration of blood cells (especially red blood cells), which develops due to bone marrow hyperplasia. In this regard, the correct analysis of the paths of the development of the disease allows us to determine the basic principles of pathogenetic treatment, which include a decrease in the number of blood cells and an impact directly on the places of their formation. This is achieved through the following treatments:

Such treatment should be accompanied by the appointment of antiplatelet drugs such as aspirin, chimes, clopidogrel or anticoagulants (heparin). The use of these drugs with one of the procedures significantly increases the effectiveness of therapy than using them separately.

It is also recommended to add some cytostatic drugs (if the cause of bone marrow hyperplasia is cancer), interferons (with the development of secondary viral complications) or hormones (mainly dexamethasone and prednisone) to the treatment regimens, which improves the prognosis of the disease.

Complications, consequences and prognosis

All complications of the disease are due to the development of vascular thrombosis. As a result of their blockage, infarcts of internal organs (heart, liver, spleen, brain), obliterating atherosclerosis (with thrombosis of vessels of the lower extremities affected by atherosclerotic plaques) can develop. An excess of hemoglobin in the blood provokes the development of hemochromatosis, urolithiasis or gout.

All of them develop secondarily and require the elimination of the main cause - erythrocytosis, for the most effective cure.

As for the prognosis of the disease, much depends on the age at which treatment was started, what methods were used, and whether they were effective.

As mentioned at the beginning, polycythemia vera tends to develop later. If the appearance of the main symptoms in young people (aged 25 to 40 years) is observed, then the disease proceeds malignantly, that is, the prognosis is unfavorable, and secondary complications develop much faster. Accordingly, the later the development of the disease is observed, the more benignly it proceeds. In the case when adequately prescribed drugs are used, the period and life of patients is significantly improved. Such patients can live normally with their disease for quite a long time (up to several decades).

Answering the question, what could be the outcome of erythremia, it should be noted that it all depends on:

• what secondary processes have developed
• what are their reasons
• how long have they existed
• whether polycythemia vera was diagnosed in a timely manner and the necessary treatment was started.

Most often, due to damage to the liver and spleen, there is a transition from polycythemia to the chronic form of myeloid leukemia. The life expectancy with it remains almost the same, and with the right selection of drugs it can reach tens of years (forecast regarding

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

The disease does not have a high prevalence - no more than five cases are recorded annually per one million population. Most often, polycythemia develops in middle-aged and elderly people. According to statistics, males suffer from this pathology five times more often than women. Today we will consider in more detail such a condition 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.

Disease classification

There are two groups of illness:

True polycythemia, or Wakez'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, climbing to a height).

Relative polycythemia (stress or false) - in this condition, the level of red blood cells remains within the normal range.

Polycythemia: symptoms of the disease

Very often the disease is asymptomatic. Sometimes, as a result of an examination for completely different reasons, polycythemia vera can be accidentally detected. The symptoms to watch out for are listed below.

Expansion of the saphenous veins

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

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

Skin itching

Almost half of patients with polycythemia develop severe itching, especially after taking a warm bath. This phenomenon acts as a specific sign of true polycythemia. Itching occurs due to the release of active substances into the blood, in particular histamine, which is able to expand 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 ​​\u200b\u200bthe fingertips. It provokes their increase in the level of platelets in the small vessels of the hand, as a result, numerous microthrombi are formed that clog arterioles and block the flow of blood to the tissues of the fingers. External signs of this condition are redness and the appearance of cyanotic spots on the skin. For the purpose it is recommended to take aspirin.

Splenomegaly (enlargement of the spleen)

In addition to the spleen, the liver can also change, or rather, its size. 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

Such a serious surgical pathology develops as a result of thrombosis of small vessels of the mucous membrane of the digestive tract. The result of an acute one is necrosis (necrosis) of a section of the organ wall and the formation of an ulcer defect in its place. In addition, the resistance of the stomach to Helicobacter (a microorganism that causes gastritis and ulcers) decreases.

Thrombi in large vessels

The veins of the lower extremities are more susceptible to such pathology. from the vessel wall, they can, bypassing the heart, enter the pulmonary circulation (lungs) and provoke PE (pulmonary embolism) - 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 can occur with polycythemia.

Gout

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

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

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, goosebumps before the eyes, shortness of breath, An increase in the viscosity of the blood activates the compensatory response of blood vessels As a result, there is an increase in 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 a 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;

elevated hemoglobin level - up to 240 g / l;

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

Since the number of erythrocytes cannot be calculated based on the measurements of the above values, radionuclide diagnostics is 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 Wakez 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 (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 TBE level drops to the desired value. If there are contraindications for bloodletting, it is possible to restore the percentage of red blood cells by diluting blood by adding a liquid part to it (high-molecular solutions are administered intravenously).

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

With such an ailment as true polycythemia, treatment involves following a certain diet. It is recommended to limit the consumption of meat and fish products, as they contain a high amount of protein, which actively stimulates the activity of the blood-forming organs. You should also avoid fatty foods. Cholesterol contributes to the development of atherosclerosis, as a result of which blood clots occur, which are already formed in large numbers in people suffering from polycythemia.

Also, if polycythemia is diagnosed, treatment may include chemotherapy. Apply it with increased thrombocytosis and severe itching. As a rule, this is a "cytoreductive agent" (drug "Hydroxycarbamide").

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

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

With this pathology, it is very rarely carried out, 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 the transferred hypoxia, which could be provoked. The baby's body begins to synthesize a large number of red blood cells to correct hypoxia.

In addition to respiratory due, newborns can also develop true polycythemia. Twins are particularly at 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 of the course: the initial stage, the stage of proliferation and depletion. Let's briefly describe them.

The initial stage of the disease practically does not have any clinical manifestations. It is possible to identify polycythemia in a child at this stage only by examining peripheral blood parameters: hematocrit, hemoglobin and red blood cells.

At the stage of proliferation, an increase in the liver and spleen develops. Plethoric phenomena are observed: the skin acquires a characteristic "plethoric-red" shade, the child shows anxiety when touching the skin. Plethoric syndrome is complemented by thrombosis. In the analyzes, there is a change in the number of erythrocytes, platelets and leukocyte shifts. The indicators of all blood cells can also increase, this phenomenon is called "panmyelosis".

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

For a newborn, such clinical changes are extremely severe and can provoke irreversible changes and subsequent death. Polycythemia can cause a malfunction in the production of certain types of white blood cells, which are responsible for the body's immune system. As a result, the infant develops severe bacterial infections, eventually leading to death.

After reading this article, you learned more about such a pathology as polycythemia. Symptoms and treatment have been considered by us in as much detail as possible. We hope that the information provided will be useful to you. Take care of yourself and be healthy!

Stage 1 - asymptomatic, duration up to 5 years or more.

Stage 2A - erythremic extended stage without myeloid metaplasia of the spleen - duration 10-20 years.

Stage 2B - erythremic with myeloid metaplasia of the spleen.

Stage 3 - posterythemic myeloid metaplasia with or without myelofibrosis.

Vascular complications in polycythemia vera .

Microvascular thrombophilic complications with clinical manifestations in the form of erythromelalgia, headache, transient visual impairment, angina pectoris.

Thrombosis of arterial and venous vessels, local and multiple.

Hemorrhages and bleeding, spontaneous and provoked by any, even small, surgical interventions.

DIC with clinical manifestations in the form of local and multiple thrombosis and bleeding (thrombotic hemorrhagic syndrome).

Criteria for the diagnosis of polycythemia vera (PVSC, USA).

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

Normal saturation of arterial blood with oxygen (more than 92%).

Splenomegaly.

Leukocytosis over 12.0x10 9 /l in the absence of infections and intoxications.

Thrombocytosis (over 400.0x10 9 /l).

The index of phosphatase activity of neutrophils is more than 100 units. (in the absence of intoxication).

Increase in unsaturated vitamin B 12 - the binding capacity of blood serum (more than 2200 pg / l).

Classification.

I. True polycythemia (erythremia).

II. Secondary absolute erythrocytosis (A, B, C).

A. Based on generalized tissue hypoxia.

1. With arterial hypoxemia.

altitude sickness,

chronic obstructive pulmonary disease,

Congenital (blue) heart defects,

Arteriovenous shunts (aneurysms) in the lungs,

Primary pulmonary hypertension, Ayers-Arrilaga disease,

Alveolar-capillary blocks of other origin,

pickwick syndrome,

Carboxyhemoglobinemia (erythrocytosis of tobacco smokers).

2. Without arterial hypoxemia:

Hemoglobinopathies with increased affinity for oxygen (hereditary erythrocytosis),

Congenital deficiency of 2,3-diphosphoglycerate in erythrocytes.

B. Paraneoblastic erythrocytosis:

kidney cancer,

Cerebellar hemangiblastoma,

Widespread hemangioblastosis (Hippel-Lindau syndrome),

hepatoma,

fibromyoma,

atrial myxoma,

Tumors of the endocrine glands,

Rarely other tumors.

C. Nephrogenic erythrocytosis (based on local hypoxia of the kidneys).

hydronephrosis,

polycystic,

stenosis of the renal arteries,

Anomaly of kidney development and other diseases.

Post-transplant erythrocytosis.

III. Relative (hemocontentration) erythrocytosis.

IV. primary erythrocytosis.

Clinical picture - In the anamnesis there are indications of pruritus associated with taking water procedures, slightly elevated red blood counts, duodenal ulcer, sometimes the first manifestations are vascular complications (erythromelalgia, vein thrombosis, necrosis of the fingers of the lower extremities, nosebleeds).

Clinical symptoms are divided into:

caused by an increase in the mass of circulating red blood cells (plethora),

caused by the proliferation of granulocytes and platelets (myeloproliferative).

An increase in the mass of circulating erythrocytes and the hematocrit index leads to an increase in blood viscosity, a slowdown in blood flow and stasis at the level of microcirculation, and an increase in peripheral vascular resistance. Erythrocyanotic coloration of the skin of the hands and face, visible mucous membranes, especially the soft palate (Kuperman's symptom) is characteristic. The limbs are hot to the touch, the patient does not tolerate heat well. The cause of splenomegaly in stage 2A is increased deposition and sequestration of blood cells, in stage 2B is the progressive development of myeloid metaplasia. An increase in the liver in stage 2A is due to increased blood supply, in stage 2B - the progressive development of myeloid metaplasia. Both stages are characterized by the development of liver fibrosis, cholelithiasis, a characteristic complication - cirrhosis of the liver. At the time of diagnosis, 35-40% of patients have arterial hypertension:

symptomatic (plethoric) hypertension associated with an increase in blood viscosity, well corrected by bloodletting,

concomitant essential hypertension aggravated by plethora,

renovascular hypertension due to sclerotic or thrombophilic stenosis of the renal arteries.

Sometimes nephrogenic hypertension develops (complication of urate diathesis and chronic pyelonephritis).

In 50-55% of patients - skin itching associated with taking water procedures. Visceral complications include ulcers/erosions of the stomach and duodenum. Violation of the metabolism of uric acid - renal colic, gout, gouty polyarthralgia.

The simultaneous tendency to hemorrhagic and thrombotic complications is a unique feature of this disease. Microcirculatory vascular diseases account for 58-80% of all complications.

Microcirculatory thrombophilic complications - erythromelalgia (attacks of acute burning pains in the fingertips of the extremities, accompanied by their sharp redness or blueness and swelling. Pain is relieved by taking aspirin.

Thrombosis of the veins of the lower extremities proceed with the clinic of thrombophlebitis, in untreated patients it is prone to recurrence, after which brown spots remain, often - melasma of the lower third of the leg, trophic ulcers.

Possible myocardial infarction, pulmonary embolism, thrombosis in the portal vein system with the development of portal hypertension.

Hemorrhagic syndrome is manifested by spontaneous bleeding of the gums, nosebleeds, ecchymosis, and massive bleeding is possible with minor surgical interventions. Thrombocytosis increases the risk of all thrombophilic complications. In 50% of patients - spontaneous platelet aggregation in the bloodstream, very often with thrombocytosis of more than 900 thousand.

Erythrocytosis causes difficulties in differential diagnosis with erythremia in cases where there is no splenomegaly, about 30% of patients do not have leukocytosis and thrombocytosis.

Differential diagnosis - measurement of the mass of circulating red blood cells (Cr 51), the volume of circulating plasma (serum albumin, labeled I 131) - with a normal mass of circulating red blood cells and a decrease in the volume of circulating plasma - the diagnosis of relative erythrocytosis. The main reason for such erythrocytosis is the intake of diuretics, smoking. Usually patients with elevated blood counts have a normal color of the skin and mucous membranes.

With an increase in the mass of circulating erythrocytes - differential diagnosis between erythremia and absolute erythrocytosis: artoxygemometry and measurement of pO 2 (several times a day). With the exclusion of arterial hypoxemia, p 50 O 2 and the oxyhemoglobin dissociation curve are determined. When it shifts to the left, hemoglobinopathy with increased affinity for oxygen or congenital deficiency of 2,3 diphosphoglycerate in erythrocytes.

In smokers, the study of carboxyhemoglobin is examined in the morning, afternoon and evening 5 days after smoking cessation.

Geisbeck's syndrome - essential arterial hypertension, overweight, neurotic personality, activation of the sympathetic-adrenal system and erythrocytosis in the blood with a normal mass of circulating erythrocytes and a decrease in the volume of circulating plasma.

With the exclusion of hypoxic erythrocytosis, the kidneys are examined, then other organs and systems.

Trepanobiopsy - about 90% informative. Neoplastic proliferation is distinguished from reactive (bleeding, sepsis, cancer of some localizations, renovascular hypertension). Rarely - changes in the bone marrow with erythremia may not be - the diagnosis is made in the process of long-term observation.

For the differential diagnosis between erythremia and symptomatic erythrocytosis, the level of erythropoietin in the blood serum and the colony-forming ability of erythroid precursors of blood and bone marrow in vitro are determined. With erythremia, the level of endogenous erythropoietin and the ability of erythroid precursors to spontaneously form colonies in culture are reduced (without the addition of erythropoietin).

Erythremia is confirmed by large forms of platelets, a violation of their aggregation properties, an increase in the number of neutrophils by more than 7 thousand, an increase in the content of alkaline phosphatase in them, a high content of IgG receptors on the neutrophil membrane, an increase in the content of lysozyme and B 12-binding protein (secretion product of neutrophils in plasma), an increase in the absolute number of basophils (stained with acrylic blue) more than 65 in 1 μl, an increase in the content of histamine in the blood and urine (secretion product of basophils)

IP outcomes - posterythremic myeloid metaplasia and myelofibrosis, transformation into acute leukemia.

Treatment of true polycythemia.

bloodletting- unloading of the vascular bed is achieved, which quickly gives a symptomatic effect, does not affect thrombocytosis and leukocytosis. Repeated bloodletting contributes to the development of iron deficiency, may be the cause of reactive thrombocytosis. Bloodletting is carried out to a hematocrit level of less than 0.45% and hemoglobin of 140-150 g/l and maintained at this level. Bloodletting is prescribed for:

benign erythremia.

its erythrocytemic variant.

reproductive age of the patient.

relapses of erythremia after cytostatic therapy with a decrease in the level of leukocytes and platelets.

Bloodletting has no leukemic effect, quickly normalizes the mass of circulating cells and blood viscosity, which prevents hemorrhagic and thrombotic complications. Bloodletting reduces itching, urate diathesis, visceral complications, has little effect on the size of the spleen, and is sometimes complicated by vascular thrombosis.

Bloodletting is carried out in a volume of 500 ml every other day in a hospital or after 2 days on an outpatient basis. In old age, with diseases of the cardiovascular system, poor tolerance - 350 ml each, the intervals between procedures are increased. On the eve of bloodletting, during the treatment period and 1-2 days after it (depending on reactive thrombocytosis), antiplatelet agents (aspirin or ticlid) are prescribed, before bloodletting - reopoliglyukin. Before bloodletting - heparin IV 5 thousand units. and 5 thousand units. x 2 times a day s / a few days after.

Then, every 6-8 weeks, control of the blood picture, with recurrence of plethoric syndrome and hemoglobin more than 140 g/l - repeated bloodletting.

With erythromelalgia(especially in the presence of thrombocytosis) - aspirin 40-80 mg daily, annually - examination by an ophthalmologist, neurologist. For the prevention of thromboembolic complications - ticlid, plavix, pentoxifylline.

Cytostatic therapy - with erythrocytosis with leukocytosis and thrombocytosis, skin itching that persists against the background of bloodletting, splenomegaly, visceral and vascular complications, a serious condition of the patient, insufficient effect of bloodletting, poor tolerance and complication of thrombocytosis, age over 50 years, inability to organize bloodletting therapy and control it.

With erythremia with thrombocythemia, young patients - hydrea orally 30 mg / kg per day in two doses for a week, then 15 mg / kg daily until leukocytosis is above 3.5 thousand, thrombocytosis is more than 100 thousand, if necessary, the maintenance dose is increased to 20 mg / kg per day.

INF-ά - 3-5 IU x 3 times a week, especially with hyperthrombocytosis.

With hyperthrombocytosis - anagrelide (affects the maturation of megakaryocytes).

Cytostatic therapy is usually combined with bloodletting.

Weekly monitoring of treatment, by the end of treatment - every 5 days. It should not be allowed to reduce leukocytes less than 5 thousand, platelets - less than 100 thousand. The results are evaluated after 2-3 months. Maintenance therapy with cytostatics is not recommended due to low efficiency, leukemia effect. Preferably timely course treatment in full or reduced volume with a tendency to relapse.

With urate diathesis, allopurinol is prescribed. In the treatment of bloodletting and cytostatics, it is prescribed prophylactically in a daily dose of 200-500 mg.

In acute vascular thrombosis - antiplatelet agents, heparin, FFP.

Prednisolone is prescribed for suspected autoimmune genesis of anemia and thrombocytopenia, in order to reduce the size of the spleen:

90-120 mg / day for 2 weeks with the transition to medium and small doses with effect and cancellation with inefficiency.

20-30 mg, then - 15-10 mg for 2-3 months with obligatory cancellation.

With posterythremic myelofibrosis, an increase in leukocytosis (more than 30 thousand), progression of splenomegaly - short courses of myelosan (4-2 mg / day for 2-3 weeks)

In the anemic stage of erythremia, splenectomy is possible:

with severe hemolytic anemia, not amenable to conservative therapy and requiring frequent transfusions.

deep thrombocytopenia with hemorrhagic syndrome with the ineffectiveness of conservative therapy.

recurrent infarcts of the spleen and mechanical compression phenomena.

extrahepatic portal block.

In postoperative thrombocytosis, antiplatelet agents are prescribed.

Prevention of vascular complications in erythremia - aspirin 40 mg / day. During the period of remission, there is no need to take drugs, except for the presence of other risk factors for vascular complications. The risk of hemorrhagic complications disappears when the hematocrit level is normalized.

In case of vascular thrombosis - aspirin 0.5-1 g for 5-7 days under control (danger of internal bleeding), simultaneously - heparin in mini-doses, fraxiparin, with a decrease in the level of ATIII during heparin therapy - FFP 400 ml intravenously by jet 1 once every 3 days, the duration of anticoagulant therapy is 1-2 weeks. With myocardial infarction, ischemic stroke, deep vein thrombosis of the thigh - thrombolytic therapy.

Treatment of microcirculatory vascular complications (erythromelalgia, angina pectoris, migraine) - aspirin - 0.3-0.5 g / day. or other disaggregants. Bleeding after tooth extraction usually stops spontaneously.

Operations for untreated erythremia are dangerous (there may be fatal hemorrhagic or thrombotic complications). If an urgent surgical intervention is necessary, the patient is prepared with the help of bloodletting and transfusion of FFP. Aspirin is canceled 7 days before any operation, with high thrombocytosis - hydrea 2-3 g / day + bleeding. For the prevention of postoperative complications - heparin in mini-doses, for patients with thrombocytosis - aspirin in small doses.

With arterial hypertension, nifedipine is poorly tolerated, they respond well to β-blockers, ACE inhibitors, and arifon.

Symptomatic therapy for pruritus - periactin (cyproheptadine) - antihistamine, antiserotonin effect, but gives a strong hypnotic effect and is poorly tolerated.

Iron-deficiency anemia- clinical and hematological syndrome, characterized by impaired hemoglobin synthesis due to iron deficiency developing due to various pathological (physiological) processes and manifested by symptoms of anemia and sideropenia.

Along with the developed symptom complex of iron deficiency anemia, there is a latent iron deficiency, characterized by a decrease in the iron content in the reserves and blood serum with normal hemoglobin levels. Latent iron deficiency is a pre-stage of iron deficiency anemia (latent anemia, "anemia without anemia") and manifests itself as an anemic syndrome with progression and lack of compensation for the iron deficiency state.

Iron deficiency anemia is the most common anemic syndrome and accounts for approximately 80% of all anemias. According to WHO (1979), the number of people with iron deficiency worldwide reaches 200 million people. The groups most vulnerable to the development of iron deficiency anemia are children of younger age groups, pregnant women and women of childbearing age.

Etiology and pathogenesis The question of the etiology of iron deficiency anemia is solved quite simply. As the name itself says, the main etiological moment of the disease is iron deficiency in the human body. However, the ways in which this deficiency occurs are very, very different: more often it is blood loss (menstrual blood loss, microblood loss from the gastrointestinal tract), an increase in the body's need for iron, which cannot be replenished by homeostatic mechanisms.

Clinical manifestations iron deficiency anemia are caused, on the one hand, by the presence of anemic syndrome, and on the other hand, by iron deficiency (hyposiderosis), to which various organs and tissues are sensitive.

The anemic syndrome is manifested by symptoms that are not specific for anemia of any origin. The main complaints of patients are reduced to weakness, fatigue, dizziness, tinnitus, flies before the eyes, palpitations, shortness of breath during exercise. The severity of the manifestations of anemia depends on the rate of decrease in hemoglobin levels and the physical activity of the patient.

sideropenic syndrome. Its clinical manifestations are associated with tissue deficiency of iron, which is necessary for the functioning of organs and tissues. The main symptomatology is observed from the skin and mucous membranes. There is dry skin, violation of the integrity of the epidermis. Ulcerations, cracks with an inflammatory shaft appear in the corners of the mouth. A typical clinical manifestation is fragility and layering of the nails, the appearance of transverse striation. Hair falls out and splits. Some patients report a burning sensation of the tongue. Taste perversions are possible in the form of an irrepressible desire to eat chalk, toothpaste, ashes, etc., as well as an addiction to certain smells (acetone, gasoline).

One of the signs of hyposiderosis is the difficulty in swallowing dry and solid food - Plummer-Vinson syndrome. In girls, less often in adult women, dysuric disorders are possible, sometimes urinary incontinence when coughing, laughing. Children may experience symptoms of nocturnal enuresis. Symptoms associated with iron deficiency include muscle weakness, associated not only with anemia, but also with a deficiency of iron-containing enzymes.

When examining patients, the pallor of the skin, often with a greenish tint, attracts attention. Hence the old name for this type of anemia is chlorosis (green). Often in patients with iron deficiency anemia, there is a distinct "blue" sclera (a symptom of blue sclera).

The main laboratory sign allowing to suspect the iron deficiency nature of anemia, is a low color indicator that reflects the hemoglobin content in the erythrocyte and is a calculated value. Since hemoglobin synthesis is impaired in iron deficiency anemia due to a lack of "building material", and the production of erythrocytes in the bone marrow decreases slightly, the calculated color index is always below 0.85, often 0.7 and below (all iron deficiency anemias are hypochromic).

The following erythrocyte indices are calculated:

The average concentration of hemoglobin in the erythrocyte (MCHC) is the ratio of Hb content in g/l to the hematocrit level in %. Normal - 30-38 g / dcl.

These indicators are analogous to the color indicator.

Average erythrocyte volume (MCV) is the ratio of Ht in 1 mm3 to the number of erythrocytes in 1 mm3 (µm3 or femtoliter - fl) or Ht in 1 mm3 x 10 and divided by the number of erythrocytes (million cells / mm3).

RDW– distribution width of erythrocytes by volume. It is calculated from the coefficient of variation of the erythrocytometric curve and expressed as a percentage. Normally 11.5-14.5%. This indicator more accurately reflects the heterogeneity of erythrocytes.

In a peripheral blood smear, hypochromic erythrocytes predominate, microcytes - the hemoglobin content in them is less than in normal-sized erythrocytes. Along with microcytosis, anisocytosis (unequal value) and poikilocytosis (various forms) of erythrocytes are noted. The number of siderocytes (erythrocytes with iron granules) is sharply reduced up to complete absence. The content of reticulocytes is within the normal range.

The content of iron in the blood serum, studied before the start of therapy with iron preparations, is reduced, often significantly. Along with the determination of serum iron, the study of the total iron-binding capacity of serum (OZHSS), which reflects the degree of "starvation" of the serum or saturation of transferrin with iron, is of diagnostic importance. In patients with iron deficiency anemia, there is an increase in TI, a decrease in the transferrin saturation coefficient.

Due to the fact that iron stores in iron deficiency anemia are depleted, there is a decrease in the serum content of ferritin, an iron-containing protein that, along with hemosiderin, reflects the amount of iron reserves in the depot.

An assessment of iron stores can be carried out by determining the iron content in the urine after the administration of certain complexons that bind iron and excrete it in the urine, in particular desferal, as well as by staining blood smears and bone marrow for iron and counting the number of siderocytes and sideroblasts. The number of these cells in iron deficiency anemia is significantly reduced.

Treatment. There are 3 stages in the treatment of iron deficiency anemia. The first stage is a cupping therapy that replenishes the level of hemoglobin and peripheral iron stores; the second is therapy that restores tissue reserves; the third is anti-relapse treatment. Pharmacy now provides a number of excellent drugs for the oral treatment of iron deficiency anemia. These include: hemostimulin, conferon, tardiferon, fenyuls, ferramid, ferro-grad-500, ferrogradment, ferrofolic-500, ferrocal, ferroplex, ferroceron, fesovit, sorbifer-durules and some others. All of them are available in capsules or in the form of tablets and dragees. As a rule, stopping therapy requires 20 to 30 days. During this time, hemoglobin is restored, the level of FBC increases, and FBSS and LZhSS decrease. However, the iron depot is not completely replenished. In this regard, a second stage of treatment, replenishing iron reserves, is necessary. This is best achieved by taking one of the above iron supplements by mouth for 3-4 months. Anti-relapse treatment consists in the periodic administration of iron preparations to patients with a high risk of recurrence of iron deficiency anemia - women with heavy and prolonged periods, other sources of blood loss, long-term breastfeeding mothers, etc.

At 12 - DEFICIENCY ANEMIA.

B12-deficiency anemia belongs to the group of megaloblastic anemias. Megaloblastic anemia is a group of diseases characterized by a weakening of DNA synthesis, as a result of which the division of all rapidly proliferating cells (hematopoietic cells, skin cells, gastrointestinal cells, mucous membranes) is disrupted. Hematopoietic cells are among the most rapidly multiplying elements, so anemia, as well as often neutropenia and thrombocytopenia, come to the fore in the clinic. The main cause of megaloblastic anemia is cyanocobalamin or folic acid deficiency.

Etiology and pathogenesis. The role of cyanocobalamin and folic acid in the development of megaloblastic anemia is associated with their participation in a wide range of metabolic processes and metabolic reactions in the body. Folic acid in the form of 5,10-methylenetetrahydrofolate is involved in the methylation of deoxyuridine, which is necessary for the synthesis of thymidine, with the formation of 5-methyltetrahydrofolate.

Cyanocobalamin is a cofactor of the methyltransferase catalytic reaction, which resynthesises methionine and simultaneously regenerates 5-methyltetrahydrofolate to tetrahydrofolate and 5,10 methylenetetrahydrofolate.

With folate and (or) cyanocobalamin deficiency, the process of incorporating uridine into the DNA of developing hematopoietic cells and the formation of thymidine is disrupted, which causes DNA fragmentation (blocking its synthesis and disruption of cell division). In this case, megaloblastosis occurs, large forms of leukocytes and platelets accumulate, their early intramedullary destruction and shortening of the life of circulating blood cells. As a result, hematopoiesis is ineffective, anemia develops, combined with thrombocytopenia and leukopenia,

In addition, cyanocobalamin is a coenzyme in the conversion of methylmalonyl-CoA to succinyl-CoA. This reaction is necessary for the metabolism of myelin in the nervous system, and therefore, with cyanocobalamin deficiency, along with megaloblastic anemia, damage to the nervous system is noted, while with folate deficiency, only the development of megaloblastic anemia is observed.

Cyanocobalamin is found in foods of animal origin - liver, kidneys, eggs, milk. Its reserves in the body of an adult (mainly in the liver) are large - about 5 mg, and given that the daily loss of the vitamin is 5 μg, then the complete depletion of reserves in the absence of intake (malabsorption, with a vegetarian diet) occurs only after 1000 days . Cyanocobalamin in the stomach binds (against the background of an acidic reaction of the environment) with an internal factor - a glycoprotein produced by the parietal cells of the stomach, or other binding proteins - K-factors present in saliva and gastric juice. These complexes protect cyanocobalamin from destruction during transport through the gastrointestinal tract. In the small intestine at an alkaline pH, under the influence of pancreatic juice proteinases, cyanocobalamin is cleaved from K-proteins and combined with intrinsic factor. In the ileum, the complex of intrinsic factor with cyanocobalamin binds to specific receptors on the surface of epithelial cells, the release of cyanocobalamin from the cells of the intestinal epithelium and transport to tissues occurs with the help of special blood plasma proteins - transcobalamins 1/2,3.

Folic acid found in green leaves of plants, fruits, liver, kidneys. Folate reserves are 5-10 mg, the minimum requirement is 50 mcg per day. Megaloblastic anemia can develop after 4 months of complete lack of dietary folate intake.

Various etiological factors can cause deficiency of cyanocobalamin or folic acid (rarely combined deficiency of both) and the development of megaloblastic anemia.

deficit cyanocobalamin may result in the following reasons:

intrinsic factor deficiency: pernicious anemia, gastrectomy, damage to the gastric epithelium by chemicals, infiltrative changes in the stomach (lymphoma or carcinoma), Crohn's disease, celiac disease, resection of the ileum, atrophic processes in the stomach and intestines,

Increased utilization of vitamin B-12 by bacteria during their overgrowth: condition after gastrointestinal anastomosis, jejunal diverticula, intestinal stasis or obstruction due to strictures,

Worm infestation: tapeworm wide,

Absorptive site pathology: ileal tuberculosis, small intestine lymphoma, sprue, regional enteritis,

Other causes: congenital absence of transcobalamin 2 (rarely), malabsorption due to the use of neomycin, colchicine.

The causes of folate deficiency can be:

1. Insufficient supply: poor diet, alcoholism, anorexia nervosa, parenteral nutrition, unbalanced nutrition in the elderly

2. Malabsorption: malabsorption, changes in the intestinal mucosa, celiac disease and sprue, Crohn's disease, regional ileitis, intestinal lymphoma, decrease in the reabsorbent surface after resection of the jejunum, taking anticonvulsants 3.Increase need: pregnancy, hemolytic anemia, exfoliative dermatitis and psoriasis

4.Disposal Violation: alcoholism, folate antagonists: trimethoprim and methotrexate, congenital disorders of folate metabolism.

The classic example of megaloblastic anemia is pernicious (B12 deficiency anemia) anemia. More often this anemia affects people older than 40-50 years.

Clinical picture: anemia develops relatively slowly and may be asymptomatic. Clinical signs of anemia are nonspecific: weakness, fatigue, shortness of breath, dizziness, palpitations. Patients are pale, subicteric. There are signs of glossitis - with areas of inflammation and atrophy of the papillae, varnished tongue, there may be an increase in the spleen and liver. Gastric secretion is sharply reduced. With fibrogastroscopy, atrophy of the gastric mucosa is detected, which is also confirmed histologically. There are also symptoms of damage to the nervous system (funicular myelosis), which do not always correlate with the severity of anemia. Neurological manifestations are based on demyelination of nerve fibers. There are distal paresthesias, peripheral polyneuropathy, sensitivity disorders, increased tendon reflexes. Thus, B 12 deficiency anemia is characterized by a triad: blood damage, gastrointestinal damage, and nervous system damage.