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.
MacQuillan
G C
GC
Liver Unit, Queen Elizabeth Hospital, 3rd Floor Nuffield House, Edgbaston, Birmingham, B15 2TH, UK.
Mutimer
D
D
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