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How is sickle cell anemia treated?
Last reviewed: 08.07.2025
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Transfusion therapy for sickle cell disease is associated with increased blood viscosity until Hb S levels are significantly reduced; hematocrit should not exceed 25-30% before initiating red blood cell transfusion. Emergency blood transfusion is indicated only when there is a need to increase blood transport function without a significant reduction in Hb S levels, such as:
- in severe anemia;
- in case of sequestration crisis;
- during aplastic crisis;
- in case of blood loss;
- before surgery.
Continuous transfusions of red blood cells are performed when it is necessary to reduce the Hb S level to less than 30% (stroke, sometimes during a severe pain crisis, pregnancy, before surgery), for this purpose red blood cells are transfused at a rate of 10-15 ml/kg every 3-4 weeks. Exchange transfusion quickly normalizes hematocrit and reduces the Hb S level. It is performed according to vital indications:
- in case of severe acute chest syndrome;
- in case of stroke;
- in arterial hypoxemia;
- refractory priapism;
- before ophthalmological surgery;
- before angiography of cerebral vessels.
When iron overload develops, chelation therapy is performed.
For a long-term reduction in the Hb S level, pharmacological stimulation of fetal hemoglobin synthesis can be used, which is effective in approximately 80% of cases. For this purpose, hydroxycarbamide (Hydrea) is prescribed at a dose of 20-30 mg/kg per day continuously, 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. With the current level of therapy, 85% of patients survive up 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 of age 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: primarily the lungs and brain.
Factors that improve the prognosis in patients with sickle cell anemia:
- Hb F level
- Hb F level > 10% provides protection against strokes;
- Hb F level > 20 % protects against pain crises and pulmonary complications.
- The presence of alpha thalassemia, which reduces the intensity of hemolysis.
- Socio-economic factors.