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

8 Citations (Scopus)

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

Fingerprint

Factor VIII
Hemophilia A
Liver
Liver Transplantation
Transplantation
Plasmas
Viruses
Fibrosis
Therapeutics
Cells
Hemorrhage
Hepatitis Delta Virus
Hepatitis C Antibodies
Esophageal and Gastric Varices
Hepatitis B virus
Hepacivirus
Surgery
HIV
Infection

Keywords

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

ASJC Scopus subject areas

  • Clinical Biochemistry

Cite this

Orthotopic liver transplantation in a patient with severe hemophilia A : a life-saving treatment for the first Italian case. / Federici, A. B.; Mannucci, P. M.; Stabile, F.; Rossi, G.; Piseddu, G.

In: International Journal of Clinical & Laboratory Research, Vol. 25, No. 1, 03.1995, p. 44-46.

Research output: Contribution to journalArticle

@article{1a33793f07444c53875504c057fc397d,
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 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.",
keywords = "Coagulation factors, Factor VIII/von Willebrand factor, Hemophilia, Liver transplantation",
author = "Federici, {A. B.} and Mannucci, {P. M.} and F. Stabile and G. Rossi and G. Piseddu",
year = "1995",
month = "3",
doi = "10.1007/BF02592576",
language = "English",
volume = "25",
pages = "44--46",
journal = "Ricerca in Clinica e in Laboratorio",
issn = "0940-5437",
publisher = "Springer Verlag",
number = "1",

}

TY - JOUR

T1 - Orthotopic liver transplantation in a patient with severe hemophilia A

T2 - a life-saving treatment for the first Italian case

AU - Federici, A. B.

AU - Mannucci, P. M.

AU - Stabile, F.

AU - Rossi, G.

AU - Piseddu, G.

PY - 1995/3

Y1 - 1995/3

N2 - 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.

AB - 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.

KW - Coagulation factors

KW - Factor VIII/von Willebrand factor

KW - Hemophilia

KW - Liver transplantation

UR - http://www.scopus.com/inward/record.url?scp=0029196566&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0029196566&partnerID=8YFLogxK

U2 - 10.1007/BF02592576

DO - 10.1007/BF02592576

M3 - Article

VL - 25

SP - 44

EP - 46

JO - Ricerca in Clinica e in Laboratorio

JF - Ricerca in Clinica e in Laboratorio

SN - 0940-5437

IS - 1

ER -