Veno-Occlusive Disease (vod) (bsh 2013)
Synonym: Sinusoidal Obstruction Syndrome (SOS)
Intro
Mean prevalence 14% depending on conditioning regimen
Severe VOD has mortality of 90% by Day +100
Diagnosis
Baltimore Criteria
Bilirubin >34 within 21 days of transplant + two of the following:
Hepatomegaly
Ascites
Weight gain >5% from pre-transplant weight
Severity
Mild = meet the criteria but do not require treatment
Moderate = not mild or severe
Severe = VOD persists beyond day +100
Risk Factors
Pre-existing hepatic dysfunction – e.g abdominal irradiation, Mylotarg, iron overload
Stem cell source, 2nd transplant, Busulfan
Underlying diagnosis – osteopetrosis, HLH, adrenoleucodystrophy
Investigations
Histopathology – occlusion of small hepatic veins
USS – reversal of portal vein flow, thickening of gallbladder wall
Prevention
Children
Defibrotide (single-stranded polydeoxyribonucleotide, unlicensed)
6.25mg/kg QDS for patients at high risk (see examples above)
Adults
Urseodeoxycholic acid (hydrophilic bile acid)
300mg BD
Treatment
Defibrotide
Fluid balance management
ITU for organ support