Hepatic veno-occlusive disease: a chemotherapy-related toxicity in children with malignancies

Palma Maurizi, Antonio Ruggiero, Maria Giuseppina Cefalo, Annalisa Arlotta, Maria Scalzone, Riccardo Riccardi, Geoffrey P. Ryan, Stanley J. Dudrick, Edward M. Copeland, Leonard R. Johnson

Risultato della ricerca: Contributo in rivistaArticolo in rivista

3 Citazioni (Scopus)

Abstract

Hepatic veno-occlusive disease (VOD) is a major manifestation of liver toxicity associated with conventional and high-dose chemotherapy in children affected by hematologic malignancies and certain solid tumors. Clinically, patients present with jaundice, painful hepatomegaly, and fluid retention, which may evolve into multi-organ failure, a hallmark of severe disease. The pathogenesis is complex and not completely understood, but the damage to sinusoidal endothelium, typically caused by toxic metabolites released from antineoplastic drugs, is thought to play a crucial role, together with cytokine activation, immune deregulation, and coagulopathy. Diagnosis is based on clinical criteria supported by characteristic ultrasound findings, with the gold standard investigation being hepatic-venous pressure gradient measurement and biopsy. Several treatment options have been tested; the most convincing approach to date is the use of defibrotide, a novel oligonucleotide with antithrombotic and antiplatelet aggregating properties, as well as endothelial-stabilizing effects. This agent, together with other specific forms of supportive care, has shown efficacy in the treatment of established VOD and promising results in the prevention of VOD in pediatric patients receiving chemotherapy
Lingua originaleEnglish
pagine (da-a)277-284
Numero di pagine8
RivistaPaediatric Drugs
Volume12
Stato di pubblicazionePubblicato - 2010

Keywords

  • chemotherapy-related
  • hepatic toxicity
  • veno-occlusive disease

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