No hepatic iron overload 12 years after liver transplantation for hereditary hemochromatosis
Authors: Marie-Pierre Bralet, Jean-Charles Duclos-Vallee, Denis Castaing, Didier Samuel, Catherine Guettier
Journal: Hepatology (2004)

To the Editor, We read with interest the results from a multinational study reported by Crawford et al.1 dealing with pathogenesis of hereditary hemochromatosis (HH) using liver transplantation model. They showed that reaccumulation of liver iron is very unusual 4 years after transplantation and that post–liver transplantation survival is reduced in HH (survival at 5 years after orthotopic liver transplantation- [OLT]: 55%). Nevertheless, half of the patients had shown excessive alcohol consumption before undergoing OLT. In our institution, 5 male patients underwent OLT for HH (mean age at OLT: 60 years) with a median follow-up of 12 years (Table 1). All patients underwent a venesection program before OLT. All were hepatitis B surface antigen negative, their alcohol consumption was below 60 g per day, and no additional cause of liver disease was detected. Three had diabetes mellitus. Indication for OLT was end-stage liver disease with cirrhosis (n = 3) or primary liver cancer (PLC) (n = 3). Two patients (patients 1 and 3) with combined hepatocellular-cholangiocarcinomas (HCK) occurring on septal fibrosis were treated with chemoembolization before OLT. One patient (patient 5) had a moderately differentiated multifocal hepatocellular carcinoma (6 nodules below 3 cm) on cirrhosis. After OLT, all 3 patients with PLC received 9 cycles of chemotherapy including 5-fluorouracil and anthracyclines. Histological iron overload was assessed using Deugnier's grading system on deparaffinized liver sections stained with Perls's stain.2 On explanted native liver, histological iron score and histological hepatic iron index are summarized on Table 1. In the latest liver biopsy post-OLT (mean histological follow up: 105 months), we never observed any iron deposit in hepatocytes, sinusoidal cells, or portal tracts. With a longer mean duration of follow-up, our data are in agreement with the study of Crawford et al. Furthermore, no patient had either abnormal serum iron, or ferritin, or transferring saturation tests after a mean follow-up period of 12.2 years. However, our survival results are quite different from those of Crawford et al. In our series, 4 patients were alive more than 8 years after OLT (ranging from 8 to 15.5 years). Moreover, we did not observe recurrence of liver tumor. Nevertheless, all patients developed extrahepatic cancers after OLT: skin cancers (porocarcinoma, basal cell carcinomas, squamous carcinomas) surgically resected, ethmoid adenocarcinoma treated with radiotherapy, and a fatal lung adenocarcinoma. In conclusion, our results showed no evidence of liver iron reaccumulation even after a long follow-up period. Although we observed a high incidence of de novo extrahepatic cancers, we emphasize the good survival after OLT for HH even when patients had primary liver cancer. Marie-Pierre Bralet M.D.*, Jean-Charles Duclos-Vallee M.D. , Denis Castaing M.D. , Didier Samuel M.D. , Catherine Guettier M.D.*, * Service d'Anatomie Pathologique Hopital Paul Brousse Villejuif, France, Centre Hepatobiliaire.