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.
Critical Care
Compendium
Chris is an Intensivist and ECMO specialist at the Alfred ICU in Melbourne. He is also a Clinical Adjunct Associate Professor at Monash University. He is a co-founder of the Australia and New Zealand Clinician Educator Network (ANZCEN) and is the Lead for the ANZCEN Clinician Educator Incubator programme. He is on the Board of Directors for the Intensive Care Foundation and is a First Part Examiner for the College of Intensive Care Medicine. He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives.
After finishing his medical degree at the University of Auckland, he continued post-graduate training in New Zealand as well as Australia’s Northern Territory, Perth and Melbourne. He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education.
He is actively involved in in using translational simulation to improve patient care and the design of processes and systems at Alfred Health. He coordinates the Alfred ICU’s education and simulation programmes and runs the unit’s education website, INTENSIVE. He created the ‘Critically Ill Airway’ course and teaches on numerous courses around the world. He is one of the founders of the FOAM movement (Free Open-Access Medical education) and is co-creator of litfl.com, the RAGE podcast, the Resuscitology course, and the SMACC conference.
His one great achievement is being the father of three amazing children.
On Twitter, he is @precordialthump.
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