A 55 year-old Chinese woman is described with severe iron overload similar in degree and distribution to that seen in hereditary hemochromatosis in the Caucasian population. Autopsy findings confirmed severe iron overload in the liver, pancreas, skin, heart, and endocrine organs. Hepatic iron concentration was 482 mumol/g with a hepatic iron index of 8.8. There was no history of thalassemia, transfusions, or alcohol abuse. Pedigree analysis revealed two HLA identical brothers that had no clinical or biochemical evidence of iron overload. This case is an unusual example of severe iron overload in a non-Caucasian kindred and may represent a non-HLA-linked form of iron overload.
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