Liver transplantation in haemophilia

Br J Haematol. 2002 Jun;117(4):952-6. doi: 10.1046/j.1365-2141.2002.03528.x.

Abstract

We report our UK single-centre experience of liver transplantation in haemophilia patients with chronic hepatitis C (HCV) infection. Between March 1990 and March 2001, 16 patients were referred for transplant assessment and 11 (mean age 46 years: nine haemophilia A, two haemophilia B) have been transplanted. Factor concentrate replacement was administered using a continuous infusion regimen following initial bolus dosing. Concentrate infusion was discontinued at a median of 36 h (range 24-72 h) post transplant. Nine patients remain alive at a median of 5 years post transplant (6 months to 11 years). One patient died 6 years post transplant from myocardial infarction. The other patient died of liver failure as a consequence of HCV infection 3 months following a second transplant, having developed HCV cirrhosis within 1 year of receiving his initial graft. Five of the seven patients who have had annual liver biopsy surveillance have developed histological changes of HCV hepatitis at a median of 3 years post transplant (1 year to 9 years). One of these patients progressed to cirrhosis at 3 years 5 months post transplant. Two patients have shown no evidence of HCV hepatitis at 2 years 8 months and 9 years post transplant respectively. The outcome of liver transplantation in haemophilic patients is good and is associated with relatively little morbidity.

MeSH terms

  • Adult
  • Blood Coagulation Factors / therapeutic use
  • Carcinoma, Hepatocellular / complications
  • Carcinoma, Hepatocellular / mortality
  • Carcinoma, Hepatocellular / surgery
  • Hemophilia A / complications*
  • Hemophilia A / mortality
  • Hemophilia A / surgery*
  • Hepatitis C, Chronic / complications*
  • Hepatitis C, Chronic / mortality
  • Hepatitis C, Chronic / surgery
  • Humans
  • Intraoperative Period
  • Liver Neoplasms / complications
  • Liver Neoplasms / mortality
  • Liver Neoplasms / surgery
  • Liver Transplantation*
  • Male
  • Middle Aged
  • Retrospective Studies
  • Survival Rate

Substances

  • Blood Coagulation Factors