Outcome of orthotopic liver transplantation in patients with haemophilia

摘要: Background—Many patients with haemophilia have developed cirrhosis or hepatocellular carcinoma due to transfusion acquired chronic viral hepatitis. Aims—To assess the long term outcome of all haemophilic reported undergone orthotopic liver transplantation. Methods—Transplant centres identified by medical database search were contacted and survival data assessed Kaplan-Meier analysis. Results—Twenty six men (median age 46 years, range 5-63 years) underwent transplantation in 16centres between 1982 1996. Indications for hepatitis C (69%), B without (15%), related (12%), biliary atresia (4%). Six (23%) infected human immunodeficiency virus (HIV). Postoperatively, median time normal clotting factor levels was 24 hours (range 0-48 hours) exogenous factors stopped at a 0-480 hours). Four (15%) had bleeding complications. The one three year HIV positive recipients (67% 23%) significantly poorer (p=0.0003) than that negative (90% 83%). Coagulopathy cured surviving more 12 days post-transplant. 20 (30%) pretransplant evidence disease recurrence mean nine months Conclusions—Hepatitis is most common indication haemophilia. Transplantation results cure but may be complicated effects infection recurrent Keywords: transplantation; haemophilia; C; cirrhosis;