Liver transplantation in haemophilia has the added bonus of, in addition to potentially cure the HCC and cirrhosis, curing the coagulation defect. However, the indications for liver transplantation are exactly the same in persons with haemophilia as in others, including the above mentioned Milan criteria for acceptable tumour load. In the study that introduced these criteria, survival was 75% at 4 years [36]. In a large multi-centre retrospective review, patients who satisfied the Milano criteria had a 5-year survival of 73%, compared to
54% in those who had larger tumours or macrovascular invasion [47]. In liver transplantation, the question is not just what the optimal treatment for an individual patient is. Given the scarcity of donor organs, the optimal use of available cadaveric livers must HDAC inhibitor also be considered. To achieve fair allocation, livers are allocated
based on objective criteria (serum bilirubin, serum creatinin, INR), which are combined Selumetinib in the Model for End-Stage Liver Disease (MELD) score [48]. The MELD score is not easily calculated, as it uses logarithms, but calculators are available online (for instance on the United Network for Organ Sharing website, http://www.unos.org). After some discussion on the relative weight of HCC, patients are now given 22 MELD points. The waiting time for transplantation is considerable, depending on blood group, local waiting list and local availability of organs. A proportion of patients has progression of HCC or dies while on the waiting list. This has prompted the use of living donor transplantation. In this procedure, the right hepatic lobe of a healthy volunteer donor (close family member or spouse) is used [49]. The advantages of a
living donor are a shorter waiting period and elective surgery. A modelling study showed that a living transplantation increases life expectancy and cost-effectiveness when compared with learn more cadaveric transplantation, as soon as the waiting time for a cadaveric transplant exceeds 7 months [50]. There is one major downside: the risk to the donor. Estimated risk of complications is 20–40% and mortality is 0.3–0.5% [49]. Evidence in haemophilia. The first successful liver transplantation in haemophilia was performed in 1985 [51]. The Birmingham haemophilia and liver centres reported a series of 11 liver transplants in haemophilia patients between 1990 and 2001. Five-year survival was nine of 11 (82%). Data on HCV recurrence were available in eight. Two developed cirrhosis at 1 and 3 years post-transplantation respectively. Four others had histological evidence of HCV hepatitis. Coagulation factor substitution was managed by continuous infusion and could be stopped at a median of 36 h after transplantation [52]. Transplantation has also been performed in patients with inhibitors to FVIII [53].