Hemochromatosis in association with hereditary spherocyto- sis

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Abstract
A case is reported of a 29-year-old woman with hereditary spherocytosis who had had a splenectomy 5 1/2 years previously, and who presented with hemochromatosis. She had skin plgmentation, amenor-rhea, portal cirrhosis with heavy iron deposition, and 99% saturation of her plasma transferrin. Removal of 17.5 g of iron by repeated phlebotomies has produced subjective improvement, increased plgmentation, and improvement in liver function tests. Family studies covering 27 blood relatives did not show any person with abnormally high serum iron levels. Possible relationships between the hereditary spherocytosis and the hemochromatosis are discussed.