Orthotopic liver transplantation in a patient with severe hemophilia A: a life-saving treatment for the first Italian case

Int J Clin Lab Res. 1995;25(1):44-6. doi: 10.1007/BF02592576.


Clinical cure of hemophilia A by orthotopic liver transplantation has been reported in 11 cases. We describe the first successful Italian case. A 27-year-old man had cirrhosis caused by previous infections with the hepatitis B, C and D viruses following life-long treatment with factor 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 severe esophageal varices required a large transfusion regimen (on average, 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 were given during surgery together with 10 bags of red cells and 11 of fresh-frozen plasma. Intraoperative bleeding was not different from that of non-hemophilic patients undergoing orthotopic liver transplantation. No additional factor VIII was used after transplantation and factor VIII levels in plasma were always above 50 U/dl, reaching the highest value of 184 U/dl on day 4 post transplantation. He was discharged from hospital 10 weeks after transplantation with factor VIII levels of 68 U/dl. All virological markers are currently negative, except anti-hepatitis C virus antibodies. In this patient orthotopic liver transplantation was a life-saving treatment for end-stage cirrhosis and a cure for hemophilia A.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Factor VIII / therapeutic use
  • Hemophilia A / surgery*
  • Humans
  • Italy
  • Liver Cirrhosis / surgery
  • Liver Failure / surgery
  • Liver Transplantation*
  • Male
  • von Willebrand Factor / therapeutic use


  • von Willebrand Factor
  • Factor VIII