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QJM 2004 97(9):581-589; doi:10.1093/qjmed/hch095
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QJM vol. 97 no. 9 © Association of Physicians 2004; all rights reserved.

Fulminant liver failure due to severe veno-occlusive disease after haematopoietic cell transplantation: a depressing experience

G.C. MacQuillan and D. Mutimer

From the Liver Unit, Queen Elizabeth Hospital, Birmingham, UK

Received 23 February 2004 and in revised form 19 May 2004

Background: Between 1988 and 2002, eight patients were referred to our unit from other institutions, for management of fulminant hepatic failure (FHF) complicating severe veno-occlusive disease (VOD).

Aim: To review our experience with these patients.

Methods: Retrospective analysis of medical case notes.

Results: In 7/8 cases, a histological diagnosis of VOD was confirmed by transjugular liver biopsy or post-mortem examination. All had undergone high-dose chemotherapy. Cyclophosphamide was included in the conditioning regimen of six patients. All developed encephalopathy and four progressed to grade 3 or 4 encephalopathy. All patients died, none surviving >75 days after haematopoietic cell transplantation. Three were listed for liver transplantation: one underwent transplantation, and two died before transplantation could be performed. Two suffered significant complications of transjugular liver biopsy. One underwent transjugular intrahepatic porto-systemic venous stent (TIPS) insertion.

Discussion: FHF complicating severe VOD is associated with multi-organ failure, and has a very poor prognosis. Our experience and that described in published literature, questions the benefits of measures such as liver transplantation or prolonged intensive care.

Address correspondence to: Dr David Mutimer, Liver Unit, Queen Elizabeth Hospital, 3rd Floor Nuffield House, Edgbaston, Birmingham, B15 2TH. e-mail: david.mutimer{at}uhb.nhs.uk


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