An autopsy case of hemochromatosis and hepatoma combined with hereditary spherocytosis.

Abstract

A case of hemochromatosisand hepatocellular carcinoma secondary to hereditary spherocytosis is very rare and this is only the second case reported in an English language bulletin. Whenthe patient, a 56 years old man, was admitted for receiving cholecystectomy for cholelithiasis, a diagnosis of hemochromatosis secondary to hereditary spherocytosis was made by liver biopsy and hematological examination data. The patient did not receive a blood transfusion nor was administered iron during the entire duration of the illness. Hepatomawas suspected at the time of splenectomy which was performed in 1981 because of severe anemia. Eight months later he died of massive abdominal cavity bleeding and subsequent autopsy findings were consistent with the disease mentioned above. Clinical and postmortem examinations suggested that conspicuously enhanced erythropoiesis in the bone marrowand unknown factors maybe responsible for an increase in iron absorption from the gut and in the amountof stored body iron, leading to the development of hemochromatosis.