Veno-Occlusive Disease (2013)

Intro

 

-       Synonym: Sinusoidal Obstruction Syndrome (SOS)

-       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:

o   Hepatomegaly

o   Ascites

o   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

 

Ix

-       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