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Home | CCC | Hepatic veno-occlusive disease

Hepatic veno-occlusive disease

by Dr Chris Nickson, last update April 24, 2019

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.

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About Dr Chris Nickson

An oslerphile emergency physician and intensivist suffering from a bad case of knowledge dipsosis. Key areas of interest include: the ED-ICU interface, toxicology, simulation and the free open-access meducation (FOAM) revolution. @Twitter | INTENSIVE| SMACC

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