Primary hemochromatosis with hereditary spherocytosis

A patient with both hereditary spherocytosis and hemochromatosis is described. At the time of the initial diagnosis of hereditary spherocytosis and shortly after splenectomy, 8 g of iron was removed by phlebotomy. During the next 15 years, the patient continued to accumulate excess iron despite splenectomy-induced remission of the hemolytic disorder. Thus the hemochromatosis in this patient was not secondary to the hereditary spherocytosis but rather represented a primary, ie, genetic abnormality of iron absorption.