Hepatic veno-occlusive disease

A chemotherapy-related toxicity in children with malignancies

Maria Giuseppina Cefalo, Palma Maurizi, Annalisa Arlotta, Maria Scalzone, Giorgio Attin, Antonio Ruggiero, Riccardo Riccardi

Research output: Contribution to journalArticle

28 Citations (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.

Original languageEnglish
Pages (from-to)277-284
Number of pages8
JournalPediatric Drugs
Volume12
Issue number5
DOIs
Publication statusPublished - 2010

Fingerprint

Hepatic Veno-Occlusive Disease
Drug Therapy
Neoplasms
Venous Pressure
Hepatomegaly
Poisons
Liver
Hematologic Neoplasms
Jaundice
Oligonucleotides
Antineoplastic Agents
Endothelium
Pediatrics
Cytokines
Biopsy
Therapeutics

Keywords

  • Antineoplastics, adverse reactions
  • Cancer
  • Children
  • Defibrotide, therapeutic use
  • Hepatic-veno-occlusive-disease, prevention
  • Hepatic-veno-occlusive-disease, treatment

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pharmacology (medical)

Cite this

Hepatic veno-occlusive disease : A chemotherapy-related toxicity in children with malignancies. / Cefalo, Maria Giuseppina; Maurizi, Palma; Arlotta, Annalisa; Scalzone, Maria; Attin, Giorgio; Ruggiero, Antonio; Riccardi, Riccardo.

In: Pediatric Drugs, Vol. 12, No. 5, 2010, p. 277-284.

Research output: Contribution to journalArticle

Cefalo, MG, Maurizi, P, Arlotta, A, Scalzone, M, Attin, G, Ruggiero, A & Riccardi, R 2010, 'Hepatic veno-occlusive disease: A chemotherapy-related toxicity in children with malignancies', Pediatric Drugs, vol. 12, no. 5, pp. 277-284. https://doi.org/10.2165/11531840-000000000-00000
Cefalo, Maria Giuseppina ; Maurizi, Palma ; Arlotta, Annalisa ; Scalzone, Maria ; Attin, Giorgio ; Ruggiero, Antonio ; Riccardi, Riccardo. / Hepatic veno-occlusive disease : A chemotherapy-related toxicity in children with malignancies. In: Pediatric Drugs. 2010 ; Vol. 12, No. 5. pp. 277-284.
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