Eritrea is what? Stages of erythremia

Erythremia is one of the types of blood diseases that is characterized by proliferation (proliferation) of erythrocytes and a noticeable increase in the number of other cells (leukocytes, platelets). It is chronic, often benign, a disease that is rare enough. However, it is possible to degenerate benign leukemia into a malignant one. Erytremia is a disease that affects about 4 people a year from 100 million people a year. It does not depend on gender, but is usually diagnosed in older and older people. Although registered cases of the disease and at a younger age, it is often women.

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

Blood cells and disease mechanism

The mechanism of erythremia develops as follows.

Erythrocytes are red blood cells, the main function of which is the transfer of oxygen to all the cells of our body. They are produced in the bone marrow, spleen and liver. Bone marrow is found in tubular bones, vertebrae, ribs, skull bones and sternum. At 96%, red blood cells are filled with hemoglobin, which performs the respiratory function. Red color is given to them by iron. Erythrocytes are formed by the stem cell. Their distinctive ability is the ability to share (multiply), transforming into any other cell.

In the bones, there is also a yellow bone marrow, which is represented by adipose tissue. He begins to develop blood elements only under extreme conditions, when the remaining sources of hematopoies do not perform their functions.

Erythremia (ICD-10 is an international classification of diseases in which this disorder is assigned code C 94.1) is a disease whose pathogenesis has not yet been studied. For reasons that are still not fully understood by the doctors, the body begins to intensively produce red blood cells, which cease to fit in the bloodstream, the blood viscosity increases, and clots begin to form. Over time, increases in hypoxia (oxygen starvation). Cells do not receive proper nutrition, there is a failure in the overall system of the body.

Some interesting facts about erythremia

1. Erytremia is the most benign disease of all leukemias. That is, for a long time it behaves passively and leads to complications rather late.
2. It can be asymptomatic and do not report yourself for many years.
3. Despite the increased level of platelets, patients are prone to heavy bleeding.
4. Erytremy has a family predisposition, so if there is a patient in the family, the risk of its appearance in one of the relatives increases.

Causes of Erythema

Like other types of leukemia, erythremia has not yet been fully studied, and the causes of its appearance have not been identified. However, the factors that are at its disposal are voiced:

1. Genetic predisposition.
2. Toxic substances in the body.
3. Ionizing radiation.

Genetic predisposition

At present, it is not known which gene mutations cause the appearance of erythremia, but it is established that in the family the disease is often repeated in the next generations. The probability of developing the disease is increased many times, if a person suffers:

• Down's syndrome (violation of the shape of the face and neck, lag in development);
• Klinefelter's syndrome (disproportionate figure and possible lag in development);
• Bloom's syndrome (low growth, pigmentation on the face and its disproportionate development, predisposition to oncological diseases);
• Marfan syndrome (disruption of the development of connective tissue).

Predisposition to erythremia is due to the fact that the genetic cell apparatus (including the blood apparatus) is unstable, so a person becomes susceptible to external negative effects - toxins, radiation.

Radiation

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

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

Toxic substances

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

• Benzene (found in gasoline and many chemical solvents);
• Antibacterial drugs (in particular, "Levomycetin");
• Cytostatic drugs (antitumor drugs).

Stages of erythremia and their symptoms

Erythremia stage has the following: initial, expanded and terminal. Each of them has its own symptoms. The initial stage can last for decades without showing any serious symptoms. Minor symptoms are usually attributed to other, less serious diseases. After all, even doctors often do not pay due attention to non-ideal blood analysis.

If there is an initial erythremia, the blood tests have moderate deviations from the norm.

The initial stage is also characterized by rapid fatigue, dizziness, noise in the ears. The patient does not sleep well, feels cold in the limbs, there are swelling of the hands and feet. There is a decrease in mental performance. There are no external manifestations of the disease yet. Headache is not considered a specific symptom of the disease, but it takes place at the initial stage due to poor blood circulation in the brain. For the same reason, vision, attention and ingenuity are reduced. When the diagnosis of "erythremia" symptoms of the disease in the second stage have their own characteristics - bleeding gums, small hematomas become noticeable. On the lower leg can appear dark spots (symptoms of thrombosis) and even trophic ulcers. The organs in which erythrocytes are formed are enlarged, - the spleen and the liver. Because of the development of the disease, lymph nodes swell.

The second stage of erythremia can last about 10 years. Weight is noticeably reduced. The skin acquires a cherry tone (more often the arms and legs), the soft palate changes color, the hard palate retains its former shade. The patient is disturbed by skin itching, which is intensified after bathing in warm or hot water. On the body, swollen veins become visible from an excess of blood, especially in the neck. Eyes like bloodshot, because erythremia, the symptoms of which are sufficiently obvious in the second stage, contributes to the inflow of blood to the vessels of the eyes.

Because of poor circulation in the capillaries, the pain and burning sensation in the fingers and toes is manifested. In neglected cases, cyanotic spots on them become noticeable.

Bone and epigastric pains occur. Joint arthritic pain is associated with excess uric acid. In general, the kidney function is disturbed, pyelonephritis is often diagnosed and urate stones in the kidneys are found.

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

The viscosity of the blood rises, which leads to the appearance of thrombi in a variety of vascular sites. There is also a risk of developing varicose veins.

Attention! Patients may also suffer from hypertension and the discovery of ulcers in the duodenum. This is due to a decrease in the defenses of the body and an increase in the number of Helicobacter pylori - it is this bacterium that causes an ulcer.

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

The third stage of erythremia: symptoms and complications

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

Previously, it was the increased thrombus formation that caused the death of patients with erythremia. The walls of the vessels are subject to changes, there is a blockage of veins in the vessels of the brain, spleen, heart and legs. There is an obliterating endarteritis - occlusion of the vessels of the legs with the risk of their complete constriction.

The kidneys are affected. Because of the growth of uric acid, patients suffer from arthritic pains of a gouty character.

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

Course of the disease

Erytremia is a slow disease. The disease comes into its own slowly, its onset is progressive and has a subtle character. Patients sometimes live for decades, not paying attention to minor symptoms. Nevertheless, in more serious cases, due to thrombi, death can occur within 4-5 years.

Along with erythremia, the spleen increases. Isolate the form with cirrhosis of the liver and defeat the intermediate brain. During the illness, allergic and infectious complications can be involved, sometimes patients do not tolerate certain groups of drugs, suffer from hives and other skin diseases. The course of the disease is complicated by concomitant conditions, because the diagnosis is usually made for the elderly.

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

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

Blood test for erythremia

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

If erythremia is diagnosed, a blood test, which indicates an increase in erythrocytes, also indicates a corresponding increase in hemoglobin. Hematocrit, indicating the ability of the blood to carry oxygen, increased to 60-80%. The color indicator is not changed, but in the third stage it can be any - normal, elevated or low. Thrombocytes and leukocytes are increased. Leukocytes grow several times, and sometimes even more. The production of eosinophils (sometimes together with basophils) is enhanced. Seriously elevated platelets. The rate of erythrocyte sedimentation is not more than 2 mm / hr.

Alas, the general analysis of the blood, although important in the initial diagnosis, is of little informative, and only on its basis is the diagnosis not made.

Other studies with erythremia

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

Treatment of erythremia

It should be noted that erythremia often progresses slowly and has a benign course. First and foremost, doctors advise to change the way of life - to be more outdoors, to walk, to receive positive emotions (endorphin therapy sometimes achieves amazing results). Products that contain a large amount of iron and vitamin C should be excluded.

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

Normalize the hematocrit with hemoglobin helps bleeding, which in this case is used to this day. However, this procedure is done as part of emergency care, because it stimulates the bone marrow and the function of thrombopoiesis (the process of platelet formation). There is also a procedure called erythrocytapheresis, which consists in purifying blood from red blood cells. In this case, the blood plasma remains.

Medication

Cytotoxic drugs are preparations of an antitumor nature that are used for complications of erythremia - ulcers, thromboses, circulatory disorders of the brain that occur in the second stage of the disease. "Myelosan", "Busulfan", hydroxyurea, "Imifos", radioactive phosphorus. The latter is considered effective due to the fact that it accumulates in the bones and depresses the function of the brain.

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

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

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

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

Erythema, often treated in a hospital, is a serious illness that causes many complications. It is important to identify it as soon as possible and begin therapy.