RAPID RE-ACCUMULATION OF HEPATIC IRON LEADING TO GRAFT FAILURE IN A LIVER TRANSPLANT RECIPIENT WITH HEMOCHROMATOSIS

Kevin Rioux, Laurence Jewell, Vincent Bain, Mang Ma

University of Alberta, Edmonton, Alberta

There is a decreased survival in the patients who underwent orthotopic liver transplantation (OLT) for hemochromatosis. There is a paucity of data regarding hepatic allograft iron accumulation in these patients. We report a case of rapid hepatic iron re-accumulation leading to hepatic graft failure.
A 36 year-old male was admitted to the University of Alberta Hospital with liver failure. Although he had a history of significant alcohol use, marked cholestasis at the time of presentation prompted an ERCP, which was suggestive of PSC. He rapidly deteriorated, necessitating liver transplantation. There were no histopathological features of PSC in the explanted liver. An unexpected finding was the presence of heavy hepatocellular iron deposition in the explanted liver (hepatic iron index of 4.0), consistent with genetic hemochromatosis. The patient did poorly after transplantation, with ongoing poor hepatic graft function, encephalopathy and persistent fever of unknown origin. Multiple liver biopsies showed no evidence of graft rejection or infection. He died 6 weeks after liver transplantation of acute respiratory failure with superimposed liver, renal, and cardiac failure. In keeping with genetic hemochromatosis, post-mortem investigation showed marked iron accumulation in the heart and, to a lesser degree, in the pancreas, spleen, kidneys, and adrenal glands. Although biopsies from the donor liver at the time of transplantation showed no appreciable iron, a remarkable finding in the post-mortem specimen was significant iron re-accumulation (hepatic iron index 2.3) after only 6 weeks.
This case frames a discussion of liver transplantation in hemochromatosis and describes a unique finding of rapid hepatic iron re-accumulation contributing to graft failure after liver transplantation.