Orthotopic liver transplantation totally corrects factor IX deficiency in hemophilia B.

Orthotopic liver transplantation has been performed for a growing range of liver-based inborn errors of metabolism. Previous authors have documented that liver transplantation can reverse the coagulation defect of hemophilia A. In this article we report total correction of factor IX deficiency in a patient with hemophilia B and blood product-related liver disease. Intraoperative bleeding was not excessive, and the donor liver produced normal amounts of factor IX immediately.