Abstract
BACKGROUND: Prior to routine screening of blood products many patients with haemophilia were infected with hepatitis C virus (HCV) and have subsequently gone on to develop end-stage liver disease (ESLD). PATIENTS AND METHODS: We report our experience of liver transplantation (LT) in patients with haemophilia that developed ESLD secondary to HCV. Patients transplanted from 1994 to 2008 were identified retrospectively. Patient demographics pre-, intra- and post-operative details and outcome were documented. RESULTS: A total of 3800 LT were performed of which 13 had haemophilia A, 4 haemophilia B and one factor (F)X deficiency. All patients were male with a median age of 52 years (range 26-59), all were HCV antibody positive, 5 (28%) were human immunodeficiency virus (HIV) positive and 4 (22%) had hepatocellular carcinoma. Median intra-operative blood loss was 4.2 l (range 0.8-12) and all received coagulation factor support peri-operatively. Coagulation was unsupported by 72 h post-operatively in all recipients. Two patients developed complications as a result of post-operative bleeding. At a median follow-up of 90 months, 8 patients have died, including 4 of the 5 patients that were HIV positive. The median survival of patients with and without HIV co-infection was 26 and 118 months, respectively. CONCLUSION: LT in patients with haemophilia cures the coagulation disorder and in the absence of HIV/HCV co-infection is associated with long-term patient survival.