title: Patient and graft survival after liver transplantation for hereditary hemochromatosis: Implications for pathogenesis.
abstract: The clinical outcome of patients who have undergone liver transplantation for hereditary hemochromatosis (HH) or who have received iron-loaded donor grafts is unclear. We reviewed 3,600 adult primary orthotopic liver transplants and assessed the outcomes in 22 patients with HH. We also evaluated graft function and iron mobilization in 12 recipients of iron-loaded donor grafts. All 22 subjects who received liver transplants for HH were male; 13 had other risk factors for liver disease. HH patients had comparatively poor outcomes following transplantation: survival at 1, 3, and 5 years posttransplantation were 72%, 62%, and 55%, respectively. Recurrent hepatocellular cancer was the most common cause of death. There was no convincing evidence of reaccumulation of iron in the grafted liver in HH; however, 1 subject demonstrated increased serum ferritin concentration and grade 2 hepatic siderosis. Liver iron stores were slow to mobilize in 7 of the 12 recipients of iron-loaded grafts. These recipients had appropriate early graft function, but 2 patients with heavy iron loading and increased hepatic iron developed hepatic fibrosis. In conclusion, (1) HH is an uncommon indication for liver transplantation, and the majority of patients requiring transplantation had other risk factors for chronic liver disease; (2) reaccumulation of liver iron in HH patients is very unusual, but increased iron stores may be slow to mobilize in normal recipients of iron-loaded grafts, potentially compromising late graft function; (3) post-liver transplant survival is reduced in HH, and affected patients require careful clinical evaluation of perioperative and postoperative risk factors. Our data suggest that iron excess in HH does not wholly depend on intestinal iron absorption but is also influenced by liver factors that moderate iron metabolism.
authors.affiliation: Queensland Liver Transplant Service and The University of Queensland, Princess Alexandra Hospital, Brisbane, Australia. darell_crawford@health.qld.gov.au
authors.forename: Darrell H G
authors.initials: DH
authors.lastname: Crawford
authors.forename: Linda M
authors.initials: LM
authors.lastname: Fletcher
authors.forename: Stefan G
authors.initials: SG
authors.lastname: Hubscher
authors.forename: Katherine A
authors.initials: KA
authors.lastname: Stuart
authors.forename: Edward
authors.initials: E
authors.lastname: Gane
authors.forename: Peter W
authors.initials: PW
authors.lastname: Angus
authors.forename: Gary P
authors.initials: GP
authors.lastname: Jeffrey
authors.forename: Geoffrey W
authors.initials: GW
authors.lastname: McCaughan
authors.forename: Paul
authors.initials: P
authors.lastname: Kerlin
authors.forename: Lawrie W
authors.initials: LW
authors.lastname: Powell
authors.forename: Elwyn E
authors.initials: EE
authors.lastname: Elias
mesh_terms: D000328:Adult; D005500:Follow-Up Studies; D006085:Graft Survival; D006432:Hemochromatosis / Q000209:etiology / Q000235:genetics* / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007501:Iron / Q000032:analysis; D008099:Liver / Q000737:chemistry; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D015996:Survival Rate; D013997:Time Factors
chemical_list: D007501:Iron