Response to Imatinib Mesylate in a Patient with Idiopathic Hypereosinophilic Syndrome Associated with Cyclic Eosinophil Oscillations

  • Shinsaku Imashuku
  • Naoki Kakazu
  • Ikuyo Ueda
  • Akira Morimoto
  • Hironori Harada
  • Tomoko Teramura
  • Shinichi Tamura
  • Yoko Fukushima-Nakase
  • Hiroshi Kuroda
Case Report

DOI: 10.1532/IJH97.04185

Cite this article as:
Imashuku, S., Kakazu, N., Ueda, I. et al. Int J Hematol (2005) 81: 310. doi:10.1532/IJH97.04185

Abstract

A 26-year-old man with idiopathic hypereosinophilic syndrome (HES) was treated with imatinib mesylate following a 5-year history of prednisolone therapy. The patient had hypereosinophilia (absolute eosinophil counts >1500/µL) occurring in cyclic oscillations as well as histologically diagnosed eosinophilic vasculitis, bursitis, and periodic soft-tissue swellings. Laboratory data revealed high levels of serum tryptase and increased numbers of mast cells in the bone marrow, but serum interleukin 5 levels were within the normal range. The disease initially responded well to 100 mg/day of imatinib mesylate but recurred 8 weeks later. Thereafter, a daily 200-mg dose was temporarily effective. Despite the response to imatinib, the FIP1L1-PDGFRA fusion gene was not detected by fluorescence in situ hybridization analysis. Additional molecular and cytogenetic studies showed neither translocations of platelet-derived growth factor receptor (PDGFR) genes nor mutations in the c-KIT or the PDGFR genes. Although imatinib mesylate is a choice of treatment for patients with HES, its precise molecular mechanism in individual cases remains to be clarified.

Key words

Idiopathic hypereosinophilic syndrome Mast cell Imatinib mesylate Tryptase Platelet-derived growth factor receptor (PDGFR) Chung-Strauss syndrome 

Copyright information

© The Japanese Society of Hematology 2005

Authors and Affiliations

  • Shinsaku Imashuku
    • 1
  • Naoki Kakazu
    • 3
  • Ikuyo Ueda
    • 4
  • Akira Morimoto
    • 4
  • Hironori Harada
    • 5
  • Tomoko Teramura
    • 1
  • Shinichi Tamura
    • 2
  • Yoko Fukushima-Nakase
    • 2
  • Hiroshi Kuroda
    • 2
  1. 1.Division of PediatricsTakasago-seibu HospitalTakasagoJapan
  2. 2.Division of PediatricsKyoto City HospitalKyoto
  3. 3.Department of Molecular-Targeting Cancer PreventionGraduate School of Medical ScienceKyoto
  4. 4.Department of PediatricsKyoto Prefectural University of MedicineKyoto
  5. 5.Department of Hematology/Oncology,Research Institute for Radiation Biology and MedicineHiroshima UniversityHiroshimaJapan

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