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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).
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Guidelines

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.
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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.
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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.
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