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Hepatic veno-occlusive disease

Guidelines

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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Screening and diagnosis

Clinical presentation: as per EASL 2016 guidelines, suspect veno-occlusive disease 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

Classification and risk stratification

Risk factors: as per BSBMT/BSH 2013 guidelines, assess patients for risk factors for veno-occlusive disease, and address these risk factors prior to HSCT.
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Diagnostic investigations

Ultrasound: as per BSBMT/BSH 2013 guidelines, obtain an abdominal ultrasound in patients with suspected veno-occlusive disease, to assist in the exclusion of other disease.
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  • Plasminogen activator inhibitor 1 levels

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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Medical management

Supportive care: as per EASL 2016 guidelines, provide supportive care for the treatment of complications of established veno-occlusive disease.
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  • Defibrotide

  • Methylprednisolone

  • Fluid balance

  • Tissue plasminogen activator

  • N-acetylcysteine

Preventative measures

Defibrotide: as per EASL 2016 guidelines, administer defibrotide to prevent veno-occlusive disease in patients undergoing HSCT.
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  • Prostaglandin E1

  • Pentoxifylline

  • UDCA

  • Anticoagulants

Follow-up and surveillance

Indications for specialist referral: as per BSBMT/BSH 2013 guidelines, obtain early consultation with critical care and hepatology specialists in patients with veno-occlusive disease, and consider other treatment options including TIPS or liver transplantation.
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