Liver transplantation for PNH with Budd-Chiari syndrome - A case report

N. Schattenforh, W. O. Bechstein, G. Blumhardt, R. Langer, H. Lobeck, J. M. Langrehr, P. Neuhaus

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24 Citations (Scopus)


A 54-year-old male patient with end-stage liver failure from Budd-Chiari syndrome due to paroxysmal nocturnal hemoglobinuria (PNH) underwent liver transplantation (OLT) in 1989. Retransplantation became necessary 1 year later when thrombotic occlusion of the portal vein and common hepatic artery led to graft loss after withdrawal of anticoagulation therapy because of several gastrointestinal bleeding episodes. The patient is now alive 3 years after the first OLT. To the best of our knowledge and according to the literature, this is, to date, the longest that any PNH patient has survived after liver transplantation. Although the course of this patient was complicated in a way similar to that reported for other cases in the literature, patients with PNH should not, in principle, be excluded from liver transplantation. Lifelong anticoagulation with coumarin and the use of steroids together with cyclosporin reduce the risk of recurrent thrombosis and PNH crises.

Original languageEnglish
Pages (from-to)354-358
Number of pages5
JournalTransplant International
Issue number6
Publication statusPublished - Nov 1993
Externally publishedYes


  • Budd-Chiari syndrome, liver transplantation
  • Hemoglobinuria, liver transplantation
  • Liver transplantation, Budd-Chiari syndrome
  • PNH, Budd-Chiari syndrome, liver transplantation

ASJC Scopus subject areas

  • Transplantation


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