Coexistence of hereditary spherocytosis and beta-thalassemia: case report of severe hemolytic anemia in an American black.

The first reported case of hereditary spherocytosis (HS) and beta-thalassemia in an American black is presented. The diagnosis rested on clinical presentation, family history and specialized laboratory findings. The tendency toward hemolysis of spherocytes and thalassemia red cells may explain the severity of anemia in this patient. The clinical course of these coexistent diseases and the role of splenectomy in relieving the hemolytic component caused by hereditary spherocytosis are described. A detailed review of the literature on HS and beta-thalassemia is also included.