title: Orthotopic liver transplantation in a patient with severe hemophilia A: a life-saving treatment for the first Italian case.
abstract: 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.
authors.affiliation: Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Institute of Internal Medicine, Milan, Italy.
authors.forename: A B
authors.initials: AB
authors.lastname: Federici
authors.forename: P M
authors.initials: PM
authors.lastname: Mannucci
authors.forename: F
authors.initials: F
authors.lastname: Stabile
authors.forename: G
authors.initials: G
authors.lastname: Rossi
authors.forename: G
authors.initials: G
authors.lastname: Piseddu
mesh_terms: D000328:Adult; D005169:Factor VIII / Q000627:therapeutic use; D006467:Hemophilia A / Q000601:surgery*; D006801:Humans; D007558:Italy; D008103:Liver Cirrhosis / Q000601:surgery; D017093:Liver Failure / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D014841:von Willebrand Factor / Q000627:therapeutic use
chemical_list: D014841:von Willebrand Factor; D005169:Factor VIII