Erythremia: what is it, symptoms, treatment, photos
Erythremia — a chronic blood disease in which the lesions observed at the cellular level. Abnormal are the cells that precede the development of myelocytes (myelopoiesis). They grow indefinitely and retain the ability to differentiate. The production of red blood cells increases. Most often affects men aged 40 to 60 years, in children almost never occurs.
Erythremia: what is it?
The so-called process of tumor formation, which is characterized by abnormalities of cell division prior to myelopoiesis. The reasons for this phenomenon are not fully understood, although it is known that disease appears because of genetic mutations. This pathology interferes with the normal regulation of growth and development of blood cells. After mutation of the cell precursor is formed of a clone, which likewise may vary in other blood cells. The problem is that these changes are not controlled by the systems of the body.
Mutant cell capable of normal reproduction, with the result that formed is functionally capable of erythrocytes. So, in the body there are normal and abnormal types of these cells. Due to the uncontrolled production of red blood cells from the cell-predecessor, the total number increases, exceeding the limit of the rules. This abnormality prevents the normal production of erythropoietin in the kidneys (substances that stimulate the transformation of normal cells-predecessors in erythrocytes) but does not affect the tumor.
Due to the increase in mutant cells all the other normal blood cells are forced out. After a certain time almost all red blood cells arise from the cells of the clone. From this clone can be formed not only erythrocytes, but all the other blood cells. This explains why in the body in eritremii is a General increase in most indicators of blood.
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Cancer or not?
Although the erythema is essentially chronic leukemia; it is characterized by a benign course. If untreated, the disease can become cancer. Then developed acute leukemia, which is characterized by a high mortality rate.
Although the causes have not been identified, there are several factors that increase the risk of developing the disease. These include:
- Down syndrome is a genetic disease is characterized by the disproportionate shape of the face, neck and head, and mental retardation.
- Bloom’s syndrome — this genetic disease is characterized by low growth, excessive pigmentation of the skin and a tendency to form tumors.
- Klinefelter syndrome — a male genetic component, which is detected only at the beginning of puberty; it is characterized by excessive growth and length of limbs, possible problems in development.
- Marfan syndrome is a genetic disease that affects connective tissue, causing tall stature, excessive length of extremities, vision problems, and cardiovascular system.
- Ionizing radiation (including uncontrolled use of x-ray diagnosis).
- Use in the treatment of chemical mutagens (some cytostatics, antibiotics, etc.).
The first signs of erythremia are excessive redness of the skin and mucous membranes across the surface of the body, pain of the fingers and toes (caused by increase blood viscosity, and oxygen starvation of tissues), headaches. With the progression of the disease the number of cells in the blood increases, it becomes thicker. The «extra» elements disrupt the coagulation (clotting) of blood and have exactly the same as normal, destroyed in the spleen. These features provoke the symptoms of the second stage.
Due to the increase of viscosity of blood the tissues of the body starve the skin becomes red, sore joints, and the fingers of the hands and feet can even undergo necrotic changes. Attempts to control systems to bring the state back to normal lead to an increase in size of liver and spleen, high blood pressure. Erythremia at this stage can cause thrombotic stroke, cardiomyopathy and myocardial infarction. The last stage to develop persistent changes, manifested by anemia and frequent bleeding. This is due to a lack of normal corpuscles. Very often the last stage eritremii ends in death.
Is it possible to cure the disease?
Since the first stage usually goes unnoticed, treatment starts only at the second stage of development of the disease. Its effectiveness was proved by leech therapy (blood-letting using leeches). Climbs up to 0.5 l of blood every few days. Thrombosis prophylaxis prescribe Aspirin or Heparin. This method is necessary for the reduction of hemoglobin and the number of gematokritnogo — volume ratio of formed elements to total blood volume.
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The ineffectiveness of this method is resorted to chemotherapy using cytotoxic drugs. These drugs have a large list of side effects, so the approach to treatment should be seriously. Chemotherapy is performed only in stationary conditions to prevent complications.
Forecast for life
Prognosis depends on timeliness of initiation of treatment, and the applied therapeutic methods. Erythremia may cause liver cirrhosis and myelofibrosis (a disease manifested by anemia of different severity). Patients with erythremia survive 10 years. New treatments prevent the transfer of disease into a malignant form, however, the disease remains chronic.
Remember that only qualified medical aid helps to prolong life and prevent complications. Do not self-medicate!