FIP1L1-PDGFRα-positive hypereosinophilic syndrome in childhood: a case report and review of literature

J Pediatr Hematol Oncol. 2014 Jan;36(1):e28-30. doi: 10.1097/MPH.0b013e31827e6386.

Abstract

Hypereosinophilic syndromes in children are rare disorders traditionally characterized by an eosinophil count exceeding 1,500/mm³ on at least 2 occasions or evidence of tissue eosinophilia associated with symptoms and marked blood eosinophilia, lacking any secondary cause (such as infections, allergic disease, chemical-induced eosinophilia, hypoadrenalism, cancer). Until now there have only been 3 reported cases of pediatric FIP1L1-PDGFRα-positive hypereosinophilic syndromes. We describe a fourth patient, a white 14-year-old boy, the third treated with imatinib.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Age of Onset
  • Antineoplastic Agents / therapeutic use
  • Benzamides / therapeutic use
  • Bone Marrow / pathology*
  • Humans
  • Hypereosinophilic Syndrome / drug therapy
  • Hypereosinophilic Syndrome / genetics*
  • Hypereosinophilic Syndrome / pathology*
  • Imatinib Mesylate
  • Male
  • Oncogene Proteins, Fusion / genetics*
  • Piperazines / therapeutic use
  • Pyrimidines / therapeutic use
  • Receptor, Platelet-Derived Growth Factor alpha / genetics*
  • mRNA Cleavage and Polyadenylation Factors / genetics*

Substances

  • Antineoplastic Agents
  • Benzamides
  • Oncogene Proteins, Fusion
  • Piperazines
  • Pyrimidines
  • mRNA Cleavage and Polyadenylation Factors
  • Imatinib Mesylate
  • FIP1L1-PDGFRA fusion protein, human
  • Receptor, Platelet-Derived Growth Factor alpha