[Hemochromatosis associated with hereditary spherocytosis]

Abstract

Hereditary spherocytosis is a chronic hemolytic anemia that very infrequently produces severe iron overload. Only 15 cases of hereditary spherocytosis associated with hemochromatosis have been described previously. It was initially thought that hemochromatosis was the result of the increase of iron stores secondary to chronic hemolysis. Afterwards, it became apparent that iron overload could appear in patients splenectomized. This fact suggested that spherocytosis and idiopathic hemochromatosis could be inherited independently. We describe the case of a 45-year-old man, with known hereditary spherocytosis, splenectomized at 5 years of age, who developed iron overload which affected his heart, liver and pancreas.