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

A. B. Federici, P. M. Mannucci, F. Stabile, G. Rossi, G. Piseddu

Research output: Contribution to journalArticle

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 trans-plantation 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 trans-plantation was a life-saving treatment for end-stage cirrhosis and a cure for hemophilia A.

Original languageEnglish
Pages (from-to)44-46
Number of pages3
JournalInternational Journal of Clinical & Laboratory Research
Volume25
Issue number1
DOIs
Publication statusPublished - Mar 1995

Keywords

  • Coagulation factors
  • Factor VIII/von Willebrand factor
  • Hemophilia
  • Liver transplantation

ASJC Scopus subject areas

  • Clinical Biochemistry

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