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


OBJECTIVE
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.


CONCLUSIONS
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.
Liver Unit, Queen Elizabeth Hospital, 3rd Floor Nuffield House, Edgbaston, Birmingham, B15 2TH, UK.
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MacQuillan
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Mutimer
D000328:Adult; D017809:Fatal Outcome; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006504:Hepatic Veno-Occlusive Disease / Q000150:complications* / Q000401:mortality; D006801:Humans; D017093:Liver Failure / Q000209:etiology* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology* / Q000401:mortality; D012189:Retrospective Studies