ORTHOTOPIC LIVER-TRANSPLANTATION IN A PATIENT WITH SEVERE HEMOPHILIA-A - A LIFESAVING TREATMENT FOR THE FIRST ITALIAN CASE

Clinical cure of hemophilia A by orthotopic liver transplantation has been reported in 11 cases. We describe the first successful Italian ca se. A 27-year-old man had cirrhosis caused by previous infections with the hepatitis B, C and D viruses following life-long treatment with f actor VIII concentrates made from large plasma pools. He was, however, seronegative for the human immunodeficiency virus. In the year before transplantation, life-threatening gastrointestinal bleeding due to se vere esophageal varices required a large transfusion regimen (on avera ge, 13 bags of red cell concentrates and 35,000 U of factor VIII/week) . To perform orthotopic liver transplantation 8,000 U of factor VIII w ere given during surgery together with 10 bags of red cells and 11 of fresh-frozen plasma. Intraoperative bleeding was not different from th at of non-hemophilic patients undergoing orthotopic liver transplantat ion. No additional factor VIII was used after transplantation and fact or VIII levels in plasma were always above 50 U/dl, reaching the highe st value of 184 U/dl on day 4 post transplantation. He was discharged from hospital 10 weeks after transplantation with factor VIII levels o f 68 U/dl. All virological markers are currently negative, except anti -hepatitis C virus antibodies. In this patient orthotopic liver transp lantation was a life-saving treatment for end-stage cirrhosis and a cu re for hemophilia A.