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; received in revised form 20 March 2010; accepted 25 March 2010. published online 31 May 2010.

Because of advances in our understanding of the hypereosinophilic syndrome (HES) and the availability of novel therapeutic agents, the original criteria defining these disorders are becoming increasingly problematic. Here, we discuss shortcomings with the current definition of HES and recent developments in the classification of these disorders. Despite significant progress in our understanding of the pathogenesis of some forms of HES, the current state of knowledge is still insufficient to formulate a new comprehensive etiologic definition of HESs. Nevertheless, we suggest a new working definition that overcomes some of the most obvious limitations with the original definition.

Key words: Definition, eosinophilia, eosinophilic leukemia, hypereosinophilic syndromes

Abbreviations used: CEL, Chronic eosinophilic leukemia, CSS, Churg-Strauss syndrome, FIP1L1, Fip1-like-1, HES, Hypereosinophilic syndrome, PDGFRA, Platelet-derived growth factor receptor α, WHO, World Health Organization

 

 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