Extrahepatic factor VIII production in transplant recipient of hemophilia donor liver.

摘要

Although the liver is the major site of production of factor VIII (FVIII), as demonstrated by correction of hemophilia A by liver transplantation,~1 extrahepatic FVIII production has been suspected ever since increased FYIII levels were recognized in end stage liver disease.~2-3 Extrahepatic FVII production has been confirmed by FVIII mRNA detection in spleen and kidneys of a pig model of fulminant liver failure,~4 the correction of hemophilia in animal models after spleen or lung transplantation,~5-6 and normal FVIII levels in normal canine recipients of hemophilia A dog livers.~7-8In humans, FVIILC levels have been detected in pulmonary endothe-lial cell supernatants.~9 Although obligate carrier donor liver transplantation corrects hemophilia,~10extrahepatic FVIII production in humans has not been demonstrated. We report normal FVIII production after transplantation of a hemophilia A donor liver into a nonhemophilic recipient with alcoholic cirrhosis.