Hereditary spherocytosis in the elderly

Five patients with hereditary spherocytosis diagnosed in their seventh to ninth decades of life are presented. These patients are remarkable for absent or mild clinical manifestations of disease. Splenectomy is the recommended treatment for hereditary spherocytosis to avoid the complications of aplastic or hemolytic crisis. When the diagnosis is made in the elderly, the treatment of choice may be careful observation with folic acid supplementation rather than splenectomy. This recommendation is based on the incidence of complications of splenectomy in the elderly in comparison to the severity and incidence of complications from the disease itself.