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, where he is Deputy Director (Education). He is a Clinical Adjunct Associate Professor at Monash University, the Lead for the Clinician Educator Incubator programme, and a CICM First Part Examiner.
He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives. He was one of the founders of the FOAM movement (Free Open-Access Medical education) has been recognised for his contributions to education with awards from ANZICS, ANZAHPE, and ACEM.
His one great achievement is being the father of three amazing children.
On Bluesky, he is @precordialthump.bsky.social and on the site that Elon has screwed up, he is @precordialthump.
| INTENSIVE | RAGE | Resuscitology | SMACC