Liver transplantation for hereditary hemochromatosis

Although the experience of orthotopic liver transplantation (OLT) for hereditary hemochromatosis (HHC) is limited, the existing data indicate that it carries a higher mortality when compared to transplantation for other causes of end-stage liver disease. Posttransplantation deaths are usually related to infectious or cardiac complications. HHC is often not diagnosed prior to OLT and one series has shown a high incidence of primary liver cancer diagnosed incidentally only at the time of transplantation. Factors that may account for the increase in postoperative mortality for HHC are the extent of iron deposition in extrahepatic sites in patients undiagnosed and thus untreated prior to transplantation. A high index of suspicion in subjects with end-stage liver disease should lead to improved diagnosis and allow for the prompt institution of either phlebotomy therapy or iron chelation therapy prior to transplantation. It is expected that these changes would reduce postoperative complications as well as improve long-term survival.