An unusual variant of hereditary spherocytosis.

Abstract

A variant of hereditary spherocytosis is described in which red cell survival studies indicated severe splenic conditioning of nearly all the circulating red cells. Clinical features also suggested a severe form of the disease with anemia, splenomegaly, a history of leg ulcers and retardation of growth in childhood. Osmotic fragility testing showed a high median corpuscular fragility which decreased appreciably after removal of the spleen. Autohemolysis was atypical for hereditary spherocytosis in that the increased hemolysis of incubated blood was not corrected by glucose supplementation, although after splenectomy the autohemolysis reverted to the typical pattern. Transfusion of the patien..