Myelodysplastic syndrome (MDS) refers to diseases of the blood system, in which normal hematopoiesis is broken. Another name for the disease - predleykemicheskoe state.
Causes of
The fact that such a myelodysplastic syndrome, and the reasons for its development is not known. Pathology is 2 types: primary (independent disease) and secondary (severe complication of processes in the human body).
Primary MDS occurs under the influence of environmental factors. These include:
- ecology;
- elevated levels of radiation;
- the action of toxins, allergens;
- the use of pesticides and other harmful substances in the cultivation of fruits and vegetables;
- adding to the foodstuff a large number of preservatives, dyes, stabilizers and others.
Other causes of primary myelodysplastic syndrome include genetic diseases. The risk group includes patients with Down syndrome, Fanconi anemia, and others.
The secondary form of the disease most often occurs on the background of malignant cancer processes, or under the influence of chemotherapeutic / radiological treatment. This leads to inhibition of germs blood (erythrocyte, leukocyte, platelet).
Classification and types
Depending on the characteristics of the disease and myelodysplastic syndromes prevalent symptoms are different. Forms of the disease according to the International Classification:
- Refractory anemia, accompanied by the appearance of sideroblasts in the blood. In the pathological process involves only erythrocyte sprout. No blasts.
- Refractory cytopenia is complicated lesion 2 shoots. Blasts do not go into the peripheral blood.
- Refractory anemia with excess blasts-1. Blasts out into the peripheral blood (5%). pancytopenia arises. In the bone marrow dysplasia processes include 1 or more of hematopoiesis germs simultaneously.
- Refractory anemia with excess blasts-2. Increased monocytes, granulocytes and lymphocytes reduced. The number of blasts increases to 19%.
- MDS is not amenable to differentiation. Decreases levels of leukocytes and platelets blasts isolated.
- MDS accompanied by an isolated deletion 5q. The analysis determined the unit blasts. Increased platelet count.
symptomatic manifestations
Specific signs have no myelodysplastic syndrome. Clinical manifestations are due to the degree of lesion of the bone marrow and inhibition of hematopoiesis germs.
The main manifestations:
- anemic syndrome. It develops mostly due to lower rates of red blood cells and hemoglobin in the blood. Of the complaints appear dizziness, severe weakness, drowsiness, palpitations, shortness of breath when walking fast, heart rhythm disturbance, decreased performance. Skin and mucous membranes become pale.
- Hemorrhagic syndrome. As a result, a sharp decline in the platelet count of blood clotting processes deteriorate. Occur spontaneous bleeding (uterine, gastrointestinal, nasal, etc.); are formed on the skin petechial hemorrhages. Even with a small impact on the skin bruises.
- infectious complications of the syndrome. Reducing the amount of mature granulocytes and violation of their operation (cells are not able to perform phagocytosis, chemotaxis, and others). It leads to the fact that the body can not effectively combat bacterial infections become susceptible to the action of pathogenic agents. Frequent complications of myelodysplastic syndrome are heavy, difficult to treat pneumonia, viral, fungal or mycobacterial nature, purulent skin lesions, blood poisoning and sepsis.
- Splenomegaly. Organ increases in sizes of 1-2 cm.
- B-symptoms. Due to changes in the number and functional capacity of lymphocytes is observed a slight increase in body temperature, especially at night. Patients reported increased fatigue, sweating, headaches and other symptoms characteristic of intoxication syndrome.
- Autoimmune syndrome. Is rare, not more than 10% of patients. Development of myelodysplastic disease leads to activation system processes: vasculitis, seronegative arthritis, and others. It is possible occurrence of pericarditis, pleurisy, hemolytic anemia.
Diagnostics
The first study, which makes it possible to suspect myelodysplastic disorder - analysis of peripheral blood. This determines a decrease of hemoglobin, erythrocytes, leukocytes (pancytopenia). Platelets can be either reduced or increased, depending on platelet destruction germ.
A distinctive feature of the disease is the high color index (0.9-1.1). This is due to the fact that the level of red blood cells falls faster than hemoglobin, causing the cells are saturated enough hemoglobin. The blood reticulocytes appear. They testify to the bone marrow an attempt to compensate for the lack of red blood cells and the exit to the periphery of immature red blood cells.
Accurate diagnosis is possible only through the study of the bone marrow biopsy from the iliac bone or sternum. The procedure is done in a hematology ward. This determines the number of blasts sideroblasts and other cells, indicating the type of pathological regeneration.
Set genetic changes help cytogenetic studies.
Treatment
Treatment of the disease - a long process. The disease therapy using the following methods and equipment:
- Symptomatic treatment. Infusions of red cell and / or platelet. Make it possible to compensate for a deficiency of form elements. If necessary frequent procedure shown desferal additional use for the purpose of prevention hemosiderosis.
- Antibiotic therapy. It shows the development of complications due to infection with the organism of bacterial microflora. In this case, use broad-spectrum antibiotics.
- Preparations for activation of cell differentiation - retinoids. These include Vesanoid, cholecalciferol, and others.
- growth factors. Is used to stimulate formation of granulocyte and monocyte cell series, as well as erythropoietin and interleukin-3 formulations.
- Immunotherapy. Gipokletochnom effective in myelodysplastic syndrome. Chemotherapy helps to normalize the blood and even achieve remission. No dose adjustment is carried out regularly on the results of blood biochemical parameters. These include creatinine, urea, liver enzymes. The treatment regimen is designed for at least 6 months.
With the development of the disease in children and young patients is shown holding a bone marrow transplant. Only this method makes it possible to achieve stable remission and prevent relapse.
Forecast
On the prognosis of the disease affects the degree of damage hematopoiesis germs (leukocyte, erythrocyte, granulocyte), a person's age and overall health. The earlier treatment starts, the more chances to achieve normalization of hematological parameters. When the timely appointment of immune therapy, therapeutic results were observed in 60% of patients.
When bone marrow transplantation for recurrence-free for 2.5-3 years, perhaps more than 35% of patients. However, there is a high mortality rate, more than 45%.
prevention
Effective prevention MDS does not exist. To avoid violations of blood, it is recommended to conduct a correct way of life. If possible, avoid contact with chemical, toxic and other harmful substances. In the food consumed organic foods are grown without the addition of pesticides and toxic chemicals. To avoid adding to food flavorings, colorants, preservatives. Consume sufficient water.
Regularly engaged in physical culture and sports. Get rid of bad habits.
If deterioration of health not to ignore health and consult a physician for a complete examination.
