Marked iron in liver explants in the absence of major hereditary hemochromatosis gene defects: A risk factor for cardiac failure

Hubert Fenton, Michael Torbenson, Perumal Vivekanandan, Matthew M. Yeh, John Hart, Linda Ferrell

Research output: Contribution to journalArticlepeer-review

19 Scopus citations

Abstract

BACKGROUND.: Patients with hereditary hemochromatosis are known to have an increased risk for morbidity and mortality after orthotopic liver transplantation. METHODS.: The clinical, histological, and genetic findings were examined in a series of seven adult patients with marked iron accumulation in their liver explants and cardiac failure despite the absence of HFE mutations. RESULTS.: Causes for cirrhosis were alcohol and hepatitis C virus (HCV) (n=2), HCV (n=1), alcohol (n=1), and cryptogenic cirrhosis (n=3). Ages at transplantation ranged from 46 to 62 years. Genetic studies confirmed all seven cases were negative for HFE mutations C282Y and H63D. The liver explants showed marked iron accumulation that predominately involved hepatocytes, with more than 90% of the iron in hepatocytes. Two patients required cardiac transplantation and four died of cardiac failure. Cardiac tissues obtained from autopsies (n=3), endomyocardial biopsy (n=1), or cardiac transplants (n=2) showed marked myocyte hypertrophy and iron deposits with or without interstitial fibrosis. CONCLUSIONS.: This study highlights a unique set of liver transplant patients with marked iron deposition in their cirrhotic liver who developed severe cardiac failure and have iron deposits in the heart, despite the absence of major HFE gene mutations. The cause of the systemic iron overload remains to be discovered.

Original languageEnglish (US)
Pages (from-to)1256-1260
Number of pages5
JournalTransplantation
Volume87
Issue number8
DOIs
StatePublished - Apr 27 2009

Keywords

  • Cardiac failure
  • Iron overload
  • Liver transplantation
  • Non-hereditary hemochromatosis

ASJC Scopus subject areas

  • Transplantation

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