ORTHOTOPIC LIVER-TRANSPLANTATION IN HEMOPHILIA-B - A CASE-REPORT

Liver transplantation is a treatment modality that is being used with increasing frequency in cases of liver-based metabolic defects. This i s a case report of a patient with hemophilia B who was treated since c hildhood with factor IX replacement for recurrent hemarthroses. Subseq uent hepatitis B (HBV) and C (HCV) infection had resulted in the devel opment of chronic active hepatitis, ultimately leading to cirrhosis. O rthotopic liver transplantation performed for endstage liver disease r esulted in a rise in factor IX:levels from 2 % to 83 % of normal value s within 24 h postoperatively, and levels remained above 90 % of norma l values after postoperative day 3 without factor IX replacement. To o ur knowledge, only two cases of hemophilia B treated by orthotopic liv er transplantation have been reported. This procedure has, however, on ly been implemented in cases of terminal liver insufficiency in hemoph iliacs.