The cause of
hemophilia is deficiency of
coagulation factor VIII production in the liver, which can be cured by
liver transplantation. Because the hepatic function of
hemophilia patients is quite normal except for production of
factor VIII, auxiliary partial orthotopic
liver transplantation (APOLT) is beneficial in that patient survival is secured by preserving native liver even in the event of graft loss. However, it is not known whether the graft of APOLT would be enough to cure
hemophilia. We evaluated the efficacy and feasibility of APOLT for
hemophilia in a canine
hemophilia A model that we established. Partial left liver graft was taken from the normal donor (blood
factor VIII activity > 60%). The graft was transplanted to the
hemophilia beagle dog (blood
factor VIII activity < 5%) after resection of the left lobe preserving native right lobe. Changes in time of blood
factor VIII activity and liver function parameters were observed after APOLT. APOLT and perioperative
hemostatic management were successfully performed. The blood
factor VIII activity increased to 30% after APOLT, and was sustained at least 6 weeks throughout the observation period without symptoms of
bleeding. The result demonstrated sustained production of
factor VIII in the
hemophilia recipient after APOLT.
Transplantation of approximately one third of whole liver resulted in cure of
hemophilia. In conclusion, it is suggested that APOLT would be feasible as a curative treatment of
hemophilia A to improve quality of life of the patients.