Idiopathic hypereosinophilic syndrome in children: report of a 7-year-old boy with FIP1L1-PDGFRA rearrangement

J Pediatr Hematol Oncol. 2005 Dec;27(12):663-5. doi: 10.1097/01.mph.0000193467.06938.77.

Abstract

Idiopathic hypereosinophilic syndrome (HES) in children is a very rare disorder; certain clinical differences with adult HES have been described, with no pediatric case with the imatinib-responsive FIP1L1-PDGFRA fusion gene reported to date. The authors describe the clinical course of three children with HES in whom FIP1L1-PDGFRA fusion gene was studied and report the first child with this rearrangement.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Anti-Inflammatory Agents / therapeutic use
  • Child
  • Female
  • Humans
  • Hypereosinophilic Syndrome / drug therapy
  • Hypereosinophilic Syndrome / epidemiology
  • Hypereosinophilic Syndrome / genetics*
  • Male
  • Oncogene Proteins, Fusion / genetics*
  • Prednisone / therapeutic use
  • Receptor, Platelet-Derived Growth Factor alpha / genetics*
  • Recurrence
  • mRNA Cleavage and Polyadenylation Factors / genetics*

Substances

  • Anti-Inflammatory Agents
  • Oncogene Proteins, Fusion
  • mRNA Cleavage and Polyadenylation Factors
  • FIP1L1-PDGFRA fusion protein, human
  • Receptor, Platelet-Derived Growth Factor alpha
  • Prednisone