Increased blood requirements during long-term transfusion therapy for sickle cell disease

Abstract

Long-term erythrocyte transfusion therapy for children with hematologic disorders often causes enlargement of the spleen and alteration of splenic function. In patients with thalassemia major, splenomegaly usually develops by 6 to 10 years of age; transfusion requirements increase because of hypersplenism as the spleen enlarges, and fall predictably after splenectomy) 3 In sickle cell disease, a disorder in which normal splenic tissue and function are usually lost in early childhood, the administration of regular erythrocyte transfusions may restore spleen size and reticuloendothelial function.4, 5 Buchanan et al. 5 suggested that continuing enlargement of the spleen in patients with sickle cell disease who regularly receive transfusions may have the clinically significant consequence of increasing blood requirements because of hypersplenism. In this report we address this hypothesis by describing four patients with sickle cell disease in whom splenomegaly and abnormally high blood requirements developed during transfusion therapy for prevention of recurrent stroke, and who have had a sustained reduction in blood requirements after splenectomy. METHODS