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Hepatic veno-occlusive disease

Key sources
The following summarized guidelines for the evaluation and management of hepatic veno-occlusive disease are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2016) and the British Society for Haematology (BSH/BSBMT 2013).


1.Screening and diagnosis

Clinical presentation: suspect VOD in patients in whom liver disease occurs in the context of prior HSCT, chemotherapy for cancer, or immunosuppression for solid organ transplantation or IBD.
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  • Diagnostic criteria

2.Classification and risk stratification

Risk factors: assess patients for risk factors for VOD, and address these risk factors prior to HSCT.

3.Diagnostic investigations

Ultrasound: obtain an abdominal ultrasound in patients with suspected VOD, to assist in the exclusion of other disease.

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  • Plasminogen activator inhibitor 1 levels

4.Diagnostic procedures

Liver biopsy: as per EASL 2016 guidelines, obtain a transjugular liver biopsy and measurement of portal venous pressures in patients who do not meet clinical criteria of sinusoidal obstruction syndrome, or when other diagnoses have to be excluded.

5.Medical management

Supportive care: provide supportive care for the treatment of complications of established VOD.

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  • Defibrotide

  • Methylprednisolone

  • Fluid balance

  • Tissue plasminogen activator

  • N-acetylcysteine

6.Preventative measures

Defibrotide: as per EASL 2016 guidelines, administer defibrotide to prevent VOD in patients undergoing HSCT.

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  • Prostaglandin E1

  • Pentoxifylline

  • UDCA

  • Anticoagulants

7.Follow-up and surveillance

Indications for specialist referral: obtain early consultation with critical care and hepatology specialists in patients with VOD, and consider other treatment options including TIPS or liver transplantation.