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

Patients with hemophilia contract hepatitis C virus (HCV) through clotting factor concentrates. Because of contamination of blood-derived clotting factor products, nearly all patients treated with large-pool donor products before the early 1990s were infected with HCV, and up to 25% of these patients developed cirrhosis. Indeed, among patients with hemophilia, endstage liver disease (ESLD) due to HCV is the leading cause of death. This population is also estimated to have an approximately 70% incidence of coinfection with human immunodeficiency virus (HIV) through the same route of transmission. It is well recognized that HIV coinfection accelerates HCV-related liver disease; thus, a large number of hemophilia patients develop ESLD, which accounts for 10% of liver transplantations for HCV/HIV-coinfected patients. With the advances in antiretroviral therapy (ART) in the mid-1990s and the improved outcome of HIV-infected patients, the indication for liver transplantation in ESLD patients with HCV/HIV coinfection is almost the same as that for HCV-monoinfected patients in all centers worldwide. Liver transplantation for hemophilia patients with ESLD due to HCV cures not only the liver failure but also coagulation abnormalities, making lifelong clotting factor replacement unnecessary. There are several reports of a relatively small number of cases of deceased donor liver transplantation (DDLT) for hemophilia patients with an acceptable outcome, but liver transplantation in these patients remains difficult with regard to the perioperative clotting factor replacement strategy and postoperative antiviral treatment for both HCV and HIV. Living donor liver transplantation (LDLT) is a mainstay for patients with ESLD in Japan, as well as for hemophilia patients. A partial liver graft in LDLT may be disadvantageous, however, in terms of the production of clotting factor. We performed 10 consecutive LDLTs for hemophilia patients with HCV-related ESLD, including 8 with HIV coinfection, which is the largest series of LDLT for hemophilia reported to date. Here we present our case series, with special reference to the clotting factor replacement strategy in LDLT for hemophilia.