Hepatic veno-occlusive disease

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.