OVERVIEW
- important complication of stem cell transplantation (SCT)
- aka sinusoidal obstruction syndrome (SOS)
- mortality >30%
CAUSES
- usually caused by the preparatory regimen given before SCT e.g. busulfan
- also seen in liver transplantation
PATHOGENESIS
- uncertain
- most likely a lesion of the sinusoidal endothelial cells of hepatic venules
- venule occlusion is not an essential feature
CLINICAL FEATURES
- weight gain
- abdominal distention
- hepatomegaly
- right upper quadrant pain
- ascites
INVESTIGATIONS
- elevated total and direct bilirubin levels
- LFT
- coag
- diagnostic ultrasound finding is a reversal of flow in the portal and hepatic veins (this may not be seen early); hepatic congestion and elevated subhepatic and portal vein pressures
MANAGEMENT
- supportive care and monitoring
- Diuretics
- Fluid restriction
- TIPS procedure for refractory ascites
- Unproven experimental treatments: thrombolytics, defibrotide (single-stranded polyribonucleotide with antithrombotic, thrombolytic, anti-ischemic and anti-inflammatory properties)
References and Links
- Coppell JA et al. Hepatic veno-occlusive disease following stem cell transplantation: incidence, clinical course, and outcome. Biol Blood Marrow Transplant. 2010 Feb;16(2):157-68. PMC3018714.
Critical Care
Compendium
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