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HPB (Oxford). 2011 Jan;13(1):40-5. doi: 10.1111/j.1477-2574.2010.00237.x. Epub 2010 Dec 1.

Outcome of liver transplantation for haemophilia.

Author information

  • 1King's College London School of Medicine at King's College Hospital, Institute of Liver Studies, King's College Hospital, Denmark Hill, London, UK.

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.

© 2010 International Hepato-Pancreato-Biliary Association.

PMID:
21159102
[PubMed - indexed for MEDLINE]
PMCID:
PMC3019540
Free PMC Article

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