The Journal of Allergy and Clinical Immunology
Volume 126, Issue 1 , Pages 45-49 , July 2010

Refining the definition of hypereosinophilic syndrome

  • Hans-Uwe Simon, MD, PhD

      Affiliations

    • Institute of Pharmacology, University of Bern, Bern, Switzerland
    • Corresponding Author InformationReprint requests: Hans-Uwe Simon, MD, PhD, Institute of Pharmacology, University of Bern, Friedbuehlstrasse 49, CH-3010 Bern, Switzerland.
    • ,
  • Marc E. Rothenberg, MD, PhD

      Affiliations

    • Division of Allergy and Immunology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio
    • ,
  • Bruce S. Bochner, MD

      Affiliations

    • Department of Medicine, Division of Allergy and Clinical Immunology, Johns Hopkins University School of Medicine, Baltimore, Md
    • ,
  • Peter F. Weller, MD

      Affiliations

    • Divisions of Allergy and Inflammation and Infectious Diseases, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Mass
    • ,
  • Andrew J. Wardlaw, MD, PhD

      Affiliations

    • Institute for Lung Health, Infection, Immunity and Inflammation, University of Leicester, Leicester, United Kingdom
    • ,
  • Michael E. Wechsler, MD

      Affiliations

    • Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, Mass
    • ,
  • Lanny J. Rosenwasser, MD

      Affiliations

    • Division of Allergy Immunology, Children's Mercy Hospital, UMKC School of Medicine, Kansas City, Mo
    • ,
  • Florence Roufosse, MD, PhD

      Affiliations

    • Hôpital Erasme, Service de Médecine Interne, Université Libre de Bruxelles, Brussels, and Institute for Medical Immunology, Université Libre de Bruxelles, Gosselies, Belgium
    • ,
  • Gerald J. Gleich, MD

      Affiliations

    • Department of Dermatology, University of Utah, Salt Lake City, Utah
    • ,
  • Amy D. Klion, MD

      Affiliations

    • Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md

Received 4 January 2010 ,Revised 20 March 2010 ,Accepted 25 March 2010.

References 

  1. Chusid MJ, Dale CD, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975;54:1–27
  2. Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes 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
  3. Klion AD, Bochner BS, Gleich GJ, Nutman TB, Rothenberg ME, Simon HU, et al. Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol. 2006;117:1292–1302
  4. Bain B, Pierre R, Imbert M, Vardiman JW, Brunning RD, Fladrin G. Chronic eosinophilic leukaemia and the hypereosinophilic syndrome. In:  Jaffe ES,  Harris NL,  Stein H,  Vardiman JW editor. World Health Organization classification of tumours: pathology and genetics of tumours of hematopoietic and lymphoid tissue. Lyon: IARC Press; 2001;p. 29–31
  5. Valent P. Pathogenesis, classification, and therapy of eosinophilia and eosinophilic disorders. Blood Rev. 2009;23:157–165
  6. Roufosse F, Cogan E, Goldman M. The hypereosinophilic syndrome revisited. Annu Rev Med. 2003;54:169–184
  7. Vassina EM, Yousefi S, Simon D, Zwicky C, Conus S, Simon HU. cIAP2 and survivin contribute to cytokine-mediated delayed eosinophil apoptosis. Eur J Immunol. 2006;36:1975–1984
  8. Simon HU, Plötz SG, Dummer R, Blaser K. Abnormal clones of T cells producing interleukin-5 in idiopathic eosinophilia. N Engl J Med. 1999;341:1112–1120
  9. Banerji A, Weller PF, Sheikh J. Cytokine-associated angioedema syndromes including episodic angioedema with eosinophilia (Gleich's syndrome). Immunol Allergy Clin North Am. 2006;26:769–781
  10. Simon D, Simon HU. Eosinophilic disorders. J Allergy Clin Immunol. 2007;119:1291–1300
  11. Plötz SG, Simon HU, Darsow U, Simon D, Vassina E, Yousefi S, et al. Use of an anti-interleukin-5 antibody in the hypereosinophilic syndrome with eosinophilic dermatitis. N Engl J Med. 2003;349:2334–2339
  12. Gevaert P, Lang-Loidolt D, Lackner A, Stammberger H, Staudinger H, van Zele T, et al. Nasal IL-5 levels determine the response to anti-IL-5 treatment in patients with nasal polyps. J Allergy Clin Immunol. 2006;118:1133–1141
  13. Simon D, Salemi S, Yousefi S, Simon HU. Primary resistance to imatinib in Fip1-like 1-platelet-derived growth factor receptor α-positive eosinophilic leukemia. J Allergy Clin Immunol. 2008;121:1054–1056
  14. Metzgeroth G, Walz C, Score J, Siebert R, Schnittger S, Haferlach C, et al. Recurrent finding of the FIP1L1-PDGFRA fusion gene in eosinophilia-associated acute myeloid leukemia and lymphoblastic T-cell lymphoma. Leukemia. 2007;21:1183–1188
  15. Capovilla M, Cayuela JM, Bilhou-Nabera C, Gardin C, Letestu R, Baran-Marzak F, et al. Synchronous FIP1L1-PDGFRA-positive chronic eosinophilic leukemia and T-cell lymphoblastic lymphoma: a bilineal clonal malignancy. Eur J Haematol. 2007;80:81–86
  16. Helbig G, Wieczorkiewicz A, Dziaczkowska-Suszek J, Majewski M, Kyrcz-Krzemien S. T-cell abnormalities are present at high frequencies in patients with hypereosinophilic syndrome. Haematologica. 2009;94:1236–1241
  17. Roufosse F, Schandené L, Sibille C, Willard-Gallo K, Kennes B, Efira A, et al. Clonal Th2 lymphocytes in patients with the idiopathic hypereosinophilic syndrome. Br J Haematol. 2000;109:540–548
  18. Simon HU, Plötz SG, Simon D, Dummer R, Blaser K. Clinical and immunological features of patients with interleukin-5-producing T cell clones and eosinophilia. Int Arch Allergy Immunol. 2001;124:242–245
  19. Roufosse F, Weller PF. Practical approach to the patient with hypereosinophilia. J Allergy Clin Immunol. 2010;126:39–44
  20. Rothenberg ME, Klion AD, Roufosse FE, Kahn JE, Weller PF, Simon HU, et al. Treatment of patients with the hypereosinophilic syndrome with mepolizumab. N Engl J Med. 2008;358:1215–1228
  21. Plager DA, Loegering DA, Checkel JL, Tang J, Kephart GM, Caffes PL, et al. Major basic protein homolog (MBP2): a specific human eosinophil marker. J Immunol. 2006;177:7340–7345
  22. De Lavareille A, Roufosse F, Schmid-Grendelmeier P, Roumier AS, Schandené L, Cogan E, et al. High serum thymus and activation-regulated chemokine levels in the lymphocytic variant of the hypereosinophilic syndrome. J Allergy Clin Immunol. 2002;110:476–479
  23. Ogbogu PU, Bochner BS, Butterfield JH, Gleich GJ, Huss-Marp J, Kahn JE, et al. Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy. J Allergy Clin Immunol. 2009;124:1319–1325
  24. Maric I, Robyn J, Metcalfe DD, Fay MP, Carter M, Wilson T, et al. KIT D816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entities. J Allergy Clin Immunol. 2007;120:680–687

 Disclosure of potential conflict of interest: H.-U. Simon has received research support from GlaxoSmithKline, AstraZeneca, Roche, CSL Behring, and Nycomed, and has provided legal consultation or expert witness testimony for Pfizer on the topic of general pharmacology. M. E. Rothenberg has consulted for and given talks for Merck; has consulted for Centocor, Ception Therapeutics, Nycomed, Array BioPharma, Biocrystal Pharmaceuticals, and Endo Pharmaceuticals; has received research support from the National Institutes of Health, the Food Allergy and Anaphylaxis Network, and the Dana Foundation; is on the medical advisory board of the American Partnership for Eosinophilic Diseases; and is on the executive council of the International Eosinophil Society. B. S. Bochner has consulted for Sanofi-Aventis and GlaxoSmithKline and has received research support from Sanofi-Aventis. P. F. Weller has received research support from Merck and has provided legal consultation services or expert witness testimony on the topic of eosinophilic diseases. A. J. Wardlaw is on advisory boards for GlaxoSmithKline and has received research support from GlaxoSmithKline, Pfizer, and AstraZeneca. M. E. Wechsler has consulted for and given talks for Genentech, Merck, and Novartis; has consulted for MedImmune, Medicinova, and GlaxoSmithKline; is on the data safety monitoring board for MAP Pharmaceuticals; and has received research support from the National Institutes of Health and GlaxoSmithKline. L. J. Rosenwasser has received research support from Novartis and Genentech and has consulted for Alcon, Sanofi-Aventis, GlaxoSmithKline, and AstraZeneca. F. Roufosse has consulted for GlaxoSmithKline. G. J. Gleich has provided legal consultation or expert witness testimony on the topic of heparin contamination and is on the board of directors for the American Partnership for Eosinophilic Disorders. A. D. Klion declares that she has no relevant conflicts of interest to disclose.

PII: S0091-6749(10)00655-X

doi: 10.1016/j.jaci.2010.03.042

The Journal of Allergy and Clinical Immunology
Volume 126, Issue 1 , Pages 45-49 , July 2010

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