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How is sickle cell anemia treated?
Last reviewed: 23.04.2024
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Transfusion treatment for sickle cell anemia is fraught with increased blood viscosity until the level of Hb S is significantly reduced; Hematocrit should not exceed 25-30% before the beginning of transfusion of erythrocyte mass. Emergency blood transfusion is indicated only if it is necessary to increase the transport function of the blood without a marked decrease in the level of Hb S, for example:
- with severe anemia;
- at sequestration crisis;
- at aplastic crisis;
- in case of blood loss;
- before surgery.
Constant transfusion of erythrocyte mass, if necessary, reduces the level of Hb S less than 30% (stroke, sometimes with severe pain, pregnancy, before surgery), for this, the erythrocyte mass is redistributed at a rate of 10-15 ml / kg every 3-4 weeks. Exchange transfusion quickly normalizes the hematocrit and reduces the level of Hb S. It is carried out according to vital indications:
- with acute thoracic syndrome of severe course;
- with stroke;
- with arterial hypoxemia;
- refractory priapism;
- before the ophthalmic operation;
- before angiography of cerebral vessels.
With the development of iron overload, chelation therapy is carried out.
For a sustained reduction in HbS, pharmacological stimulation of fetal hemoglobin synthesis, effective in about 80% of cases, can be used. To this end, prescribe hydroxycarbamide (hydrea) at a dose of 20-30 mg / kg per day constantly, the effect is dose-dependent.
In addition, bone marrow transplantation is used.
[Sickle-cell anemia prognosis
The life expectancy of patients depends on the severity of the clinical manifestations of the disease and the existing complications. At the current level of therapy, 85% of patients survive to 20 years.
The main causes of death of patients:
- Infections: sepsis, meningitis. The risk of developing sepsis or meningitis in children under 5 years is more than 15%, the mortality rate in this group reaches 30%.
- Organ failure: damage to the heart, liver, kidneys.
- Thrombosis of the vessels of vital organs: first of all, the lungs and the brain.
Factors that improve the prognosis in patients with sickle cell anemia:
- Level Hb F
- level Hb F> 10% provides protection from strokes;
- level Hb F> 20 % protects against painful crises and pulmonary complications.
- The presence of alpha-thalassemia, which reduces the intensity of hemolysis.
- Socio-economic factors.