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