Successful liver transplantation in a patient with Budd-Chiari syndrome caused by homozygous factor V Leiden

Henkie P. Tan, Jay S. Markowitz, Warren R. Maley, William T Merritt, Andrew S. Klein

Research output: Contribution to journalArticle

Abstract

Budd-Chiari syndrome (BCS) is a rare form of portal hypertension characterized by hepatic venous outflow obstruction. Although hematologic disorders are the most common cause of this syndrome, to date, 30% of the cases have been classified as idiopathic. Resistance to activated protein C caused by factor V Leiden is the most common cause of thrombophilia; its role in the pathogenesis of BCS is now becoming apparent. We report successful liver transplantation in a patient with BCS caused by homozygous factor V Leiden. The patient was administered standard heparin anticoagulation until activated protein C resistance was normalized by the liver allograft. Liver transplantation corrected the thrombophilic state. The patient has excellent graft function, is not on anticoagulation therapy, and has had no recurrent venous thrombosis at 5 months posttransplantation. Activated protein C resistance caused by the factor V Leiden mutation may be responsible for idiopathic cases of BCS. To avoid unnecessary long-term anticoagulation after liver transplantation, factor V Leiden should be considered as a pathogenic factor in BCS. In addition, because of the high prevalence of factor V Leiden in the world population, cadaveric organ donors with a history of venous thrombosis should be screened for activated protein C resistance lest thrombophilia be transmitted to the recipient.

Original languageEnglish (US)
Pages (from-to)654-656
Number of pages3
JournalLiver Transplantation
Volume6
Issue number5
StatePublished - 2000

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Budd-Chiari Syndrome
Liver Transplantation
Activated Protein C Resistance
Thrombophilia
Venous Thrombosis
R Factors
Portal Hypertension
Allografts
Heparin
factor V Leiden
Tissue Donors
Transplants
Mutation
Liver
Population

ASJC Scopus subject areas

  • Surgery
  • Transplantation

Cite this

Successful liver transplantation in a patient with Budd-Chiari syndrome caused by homozygous factor V Leiden. / Tan, Henkie P.; Markowitz, Jay S.; Maley, Warren R.; Merritt, William T; Klein, Andrew S.

In: Liver Transplantation, Vol. 6, No. 5, 2000, p. 654-656.

Research output: Contribution to journalArticle

Tan, Henkie P. ; Markowitz, Jay S. ; Maley, Warren R. ; Merritt, William T ; Klein, Andrew S. / Successful liver transplantation in a patient with Budd-Chiari syndrome caused by homozygous factor V Leiden. In: Liver Transplantation. 2000 ; Vol. 6, No. 5. pp. 654-656.
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AU - Klein, Andrew S.

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