Liver transplantation in the haemophilia patient

Abstract

Hepatitis C is a chronic condition that many persons with haemophilia contracted in the 1980s due to the infusion of factor concentrates which did not have viral inactivation processes in place. Many patients with haemophilia are now living longer lives, well into 80 years of age, due to the improvement of their care. The effects of the HCV on the liver over time are now being realized as this population ages. Although the new treatments for hepatitis C have a prolonged response, as demonstrated by a persistent negative viral load, many haemophilia patients have either not responded to the therapy or had significant side-effects to the treatment, preventing continued therapy. Of these infected haemophiliacs with liver disease, many have demonstrated a slow progressive decline resulting in liver failure, cirrhosis or liver cancer. Liver transplant then becomes their only option. This article will review liver transplantation in the haemophilia patient highlighting three case studies demonstrating the effectiveness of specific short-term factor infusions and other haemostatic support to minimize bleeding during the surgical period. These cases confirm the opportunity for successful liver transplantation for haemophilia patients utilizing specific factor recommendations with minimal bleeding risk.

Cancer detection and management in patients with haemophilia: a retrospective European multicentre study

“…Of our series' 12 HCC patients, eight underwent surgery, including four liver transplantations, without any bleeding complications. These results confirm the procedure's safety, as previously reviewed . Other transplantations, such as autologous stem cell transplantation and cord blood haematopoietic stem cell transplantation, were reported not to be associated with bleeding complications .…”

Living donor liver transplantation for hemophilia with special reference to the management of perioperative clotting factor replacement

“…On the other hand, excessive administration of the clotting factor may impair the hemostatic rebalance, resulting in thrombosis, which has a higher rate in LDLT than in DDLT. There are several reports of patient series demonstrating the perioperative clotting factor replacement in DDLT for hemophilia patients, 2,3 in which the combination of the fixed bolus injection (ie, [100-baseline] 3 BW 3 0.5) and maintenance continuous infusion (2 IU/kg/hour) with meticulous monitoring of both coagulation panels and clotting factor level are recommended. According to these reports, the intraoperative hemostasis was well controlled, and continuous infusion was commonly discontinued within 24 hours with sufficient production of the clotting factor by the graft.…”

Hepatitis C infection and outcomes in the Scottish haemophilia population

“…Indications for liver transplantation in PWH with HCV and outcomes are the same as for other groups of HCV‐infected patients when adequate coagulation factor replacement is provided . Eleven patients in this cohort have successfully undergone OLT, 2 were HIV coinfected.…”