Anemia falciforme e infecções

Abstract

Sickle cell anemia, or drepanocytosis, is a congenital structural hemoglobinopathy that has become increasingly prevalent in Spain over the past decade. It is characterized by sickleshaped red blood cells produced as a resuit of the formation of hemoglobin S polymers in the cell. This leads to the development of hemolytic anemia and thrombosis. The most common clinical signs are episodes of bone pain associated with vessel occlusion, stroke, pulmonary infiltrates, splenic sequestration, functional asplenia and multiple organ dysfunction. Treatment is based on health education focusing on the recognition of the warning signs, vaccination against encapsulated bacteria, penicillin prophylaxis, transfusions in selected situations, stimulators of fetal hemoglobin and, in severe cases, transplantation of hematopoietic progenitors from a compatible sibling.