Compound load of copper and iron in the livers of pretreatment patients with Wilson's disease

Abstract

Ceruloplasmin is a major ferroxidase in sera, and people with hypoceruloplasminemia might have iron load complications. We used histochemical and X-ray microanalysis techniques on pretreatment liver specimens from five patients with Wilson's disease. Serum levels of ceruloplasmin and ferritin were determined before chelation therapy. All the patients showed hypoceruloplasminemia ranging from 15 to 160 mg/L. Histo-chemical copper was positively stained in four of the five patients. Copper deposits were clearly observed in the hepatocyte lysosomes of one patient. High serum ferritin levels ranging from 188 to 400 ng/mL were found. Histochemical iron was positive in the livers of two patients. Iron-specific X-rays were positive in the hepatocyte lysosomes of all the patients. These findings suggest that compound load of copper and iron is a fundamental finding in the liver of Wilson's disease with hypoceruloplasminemia. Copper cytotoxicity is a primary target for chelation therapy, and iron load, if hepatotoxic, should be treated by phlebotomy. J. Trace Elem. Exp. 17:65–73, 2004. © 2004 Wiley-Liss, Inc.