Successful Treatment of Idiopathic Hypereosinophilic Syndrome with Imatinib Mesylate: A Case Report

Case Report


Patients with idiopathic hypereosinophilic syndrome (HES) show persistent hypereosinophilia of unknown etiology that is associated with end-organ damage. Different treatments, including the use of corticosteroids and cytotoxics, have been investigated for HES with modest success. We describe a patient with HES who had significant end-organ damage from hypereosinophilia and remained refractory to conventional therapy. Therapy with imatinib mesylate, a selective tyrosine kinase inhibitor that is highly effective in treating patients with BCR-ABL-positive chronic myeloid leukemia, was tried with the patient. The result was impressive, with hematologic remission achieved after 12 days of administration. Our finding concurs with recent reports that imatinib mesylate may be a promising agent in the treatment of some cases of HES.

Key words

Hypereosinophilic syndrome Imatinib mesylate Gleevec Eosinophilia 


  1. 1.
    Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. 1994;83:2759–2779.PubMedGoogle Scholar
  2. 2.
    Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975;54:1–27.CrossRefGoogle Scholar
  3. 3.
    Gleich GJ, Leiferman KM, Pardanani A, Tefferi A, Butterfield JH. Treatment of hypereosinophilic syndrome with imatinib mesilate. Lancet. 2002;359:1577–1578.PubMedCrossRefGoogle Scholar
  4. 4.
    Cortes J, Ault P, Koller C, et al. Efficacy of imatinib mesylate in the treatment of idiopathic hypereosinophilic syndrome. Blood. 2003; 101:4714–4716.PubMedCrossRefGoogle Scholar
  5. 5.
    Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003;348:1201–1214.PubMedCrossRefGoogle Scholar
  6. 6.
    Apperley JF, Gardembas, Melo JV, et al. Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. N Engl J Med. 2002;347:481–487.PubMedCrossRefGoogle Scholar
  7. 7.
    Bain BJ. Eosinophilic leukaemias and the idiopathic hypereosinophilic syndrome. Br J Haematol. 1996;95:2–9.PubMedGoogle Scholar
  8. 8.
    Flaum MA, Schooley RT, Fauci AS, Gralnick HR. A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome, I: hematologic manifestations. Blood. 1981;58:1012–1020.PubMedGoogle Scholar
  9. 9.
    Parrilo JE, Fauci AS, Wolff SM. Therapy of the hypereosinophilic syndrome. Ann Intern Med. 1978;89:167–179.Google Scholar
  10. 10.
    Parillo JE, Lawley TJ, Frank MM, Kaplan AP, Fauci AS. Immunologic reactivity in the hypereosinophilic syndrome. J Allergy Clin Immunol. 1979;64:113–121.CrossRefGoogle Scholar
  11. 11.
    Izumi T, Kishimoto S, Takano T, et al. Expression of human platelet-activating factor receptor gene in EoL-1 cells following butyrate-induced differentiation. Biochem J. 1995;305:829–835.PubMedGoogle Scholar
  12. 12.
    Seguchi M, Nakajima S. Induction of ICAM-1 expression on the eosinophilic leukemia cell line-3 (EoL-3) by platelet-activating factor and platelet factor 4. Ann Allergy Asthma Immunol. 1995;74:255–257.PubMedGoogle Scholar
  13. 13.
    Spry CJ, Davies J, Tai PC, Olsen EG, Oakley CM, Goodwin JF. Clinical features of fifteen patients with the hypereosinophilic syndrome. Q J Med. 1983;52:1–22.PubMedGoogle Scholar
  14. 14.
    Smit AJ, van Essen LH, de Vries EG. Successful long-term control of idiopathic hypereosinophilic syndrome with etoposide. Cancer. 1991;67:2826–2827.PubMedCrossRefGoogle Scholar
  15. 15.
    Murphy PT, Fennelly DF, Stuart M, O'Donnell JR. Alfa-interferon in a case of hypereosinophilic syndrome. Br J Haematol. 1990;75:619–620.PubMedCrossRefGoogle Scholar
  16. 16.
    Zilinsky RM, Lawrence WD. Interferon-a for the hypereosinophilic syndrome. Ann Intern Med. 1990;113:716–718.Google Scholar
  17. 17.
    Archimbaud E, Guyotat D, Guillaume C, Godard J, Fiere D. Hypereosinophilic syndrome with multiple organ dysfunction treated by allogeneic bone marrow transplantation. Am J Hematol. 1988;27:302–303.PubMedCrossRefGoogle Scholar
  18. 18.
    Esteva-Lorenzo FJ, Meechan KR, Spitzer TR, Mazumder A. Allogeneic bone marrow transplant in a patient with hypereosinophilic syndrome. Am J Hematol. 1996;51:164–165.PubMedCrossRefGoogle Scholar
  19. 19.
    Sigmund DA, Flessa HC. Hypereosinophilic syndrome: successful allogeneic bone marrow transplantation. Bone Marrow Transplant. 1995;15:647–648.PubMedGoogle Scholar
  20. 20.
    Fukushima T, Kuriyama K, Ito H, et al. Successful bone marrow transplantation for idiopathic hypereosinophilic syndrome. Br J Haematol. 1995;90:213–215.PubMedCrossRefGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2004

Authors and Affiliations

  • D. Tan
    • 1
  • W. Hwang
    • 1
  • H. J. Ng
    • 1
  • Y. T. Goh
    • 1
  • P. Tan
    • 1
  1. 1.Department of HaematologySingapore General HospitalOutram RdSingapore

Personalised recommendations