Liver transplantation in haemophilia

Abstract

Summary. 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.

Liver tissue engineering at extrahepatic sites in mice as a potential new therapy for genetic liver diseases

“…Because the liver is a major contributory organ for factor VIII production, 31 recent experience in transplanting whole or part of the liver into hemophilia patients have resulted in curative levels (Ͼ50%) of factor VIII production and clotting activity. 32,33 However, raising these levels into the range of 2% to 3% will result in a significant phenotypic improvement in the disease, and this prompted us to establish whether engineering liver tissues would have a therapeutic benefit on hemophilia A. 34 As shown in this study, by engineering liver tissue with 3 ϫ 10 6 hepatocytes that are equivalent to approx 3% of the naïve liver, we could demonstrate therapeutic efficacy in reconstituting 5% to 10% of normal clotting activity, enough to reduce the bleeding time to near normal values.…”

Thromboelastography Use in the Perioperative Transfusion Management of a Patient with Hemophilia A Undergoing Liver Transplantation

“…Liver transplantation in hemophiliacs reverses hepatic failure and corrects coagulation defects but not genetic transmissibility. End stage liver disease combined with hemophilia exposes the patient to a greater risk of bleeding complications during OLT, although, these patients can be safely brought through OLT without excessive transfusion requirements, using appropriate replacement regimens [3].…”

Long-term outcome of liver transplantation in HCV/HIV coinfected haemophilia patients

“…The same group achieved the first successful OLT three years later (5). At the time of writing, only about 115 haemophilia patients worldwide have undergone liver transplantation for HCV associated liver disease (1,2,4,6,7).…”

“…A HCV/HIV coinfection increases the estimated risk of end stage liver disease (ESLD) to 17% by 10 years, and finally 30% of these patients develop cirrhosis or hepatocellular carcinoma (2). From 1993-2013 in our haemophilia centre…”