« Previous Next » The Journal of Allergy and Clinical Immunology
Volume 124, Issue 6 , Pages 1319-1325.e3 , December 2009

Hypereosinophilic syndrome: A multicenter, retrospective analysis of clinical characteristics and response to therapy

Princess U. Ogbogu, MD
- National Institute of Allergy and Infectious Diseases, Laboratory of Parasitic Diseases, National Institutes of Health, Bethesda, Md
Bruce S. Bochner, MD
- Division of Allergy and Clinical Immunology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Md
Joseph H. Butterfield, MD
- Division of Allergic Diseases, Mayo Clinic, Rochester, Minn
Gerald J. Gleich, MD
- Department of Medicine, University of Utah, Salt Lake City, Utah
Johannes Huss-Marp, MD
- ZAUM—Center for Allergy and Environment, Division of Environmental Dermatology and Allergy, Helmholtz Center Munich/Technische Universität München, Munich, Germany
- Department of Dermatology and Allergy Biederstein, Technische Universität München, Munich, Germany
Jean Emmanuel Kahn, MD
- Department of Internal Medicine, Hôpital Foch, Suresnes, France
- French Eosinophil Network, Department of Immunology, CHRU de Lille, Lille, France
Kristin M. Leiferman, MD
- Department of Dermatology, University of Utah, Salt Lake City, Utah
Thomas B. Nutman, MD
- National Institute of Allergy and Infectious Diseases, Laboratory of Parasitic Diseases, National Institutes of Health, Bethesda, Md
Florian Pfab, MD
- ZAUM—Center for Allergy and Environment, Division of Environmental Dermatology and Allergy, Helmholtz Center Munich/Technische Universität München, Munich, Germany
- Department of Dermatology and Allergy Biederstein, Technische Universität München, Munich, Germany
Johannes Ring, MD
- Department of Dermatology and Allergy Biederstein, Technische Universität München, Munich, Germany
Marc E. Rothenberg, MD, PhD
- Division of Allergy and Immunology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
Florence Roufosse, MD
- Département de Médecine Interne, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium
- Institute for Medical Immunology, Université Libre de Bruxelles, Gosselies, Belgium
Marie-Helene Sajous, MD
- Division of Allergy and Clinical Immunology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Md
Javed Sheikh, MD
- Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Mass
Dagmar Simon, MD
- Department of Dermatology, Inselspital, Bern, Switzerland
Hans-Uwe Simon, MD, PhD
- Institute of Pharmacology, University of Bern, Bern, Switzerland
Miguel L. Stein, MD
- Division of Allergy and Immunology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
Andrew Wardlaw, MD
- Institute for Lung Health, University of Leicester, Leicester, United Kingdom
Peter F. Weller, MD
- Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Mass
Amy D. Klion, MD
- National Institute of Allergy and Infectious Diseases, Laboratory of Parasitic Diseases, National Institutes of Health, Bethesda, Md
- Reprint requests: Amy D. Klion, MD, Bldg 50, Rm 6351, Laboratory of Parasitic Diseases, NIAID, National Institutes of Health, Bethesda, MD 20892.

Received 13 May 2009 ,Revised 26 August 2009 ,Accepted 3 September 2009.

Clinical manifestations of HES. The clinical manifestations at initial presentation (A) and at the time of the retrospective analysis (B) are shown as the percentage of patients with evidence of organ

Clinical manifestations of HES. The clinical manifestations at initial presentation (A) and at the time of the retrospective analysis (B) are shown as the percentage of patients with evidence of organ involvement referable to a given category. Reported manifestations in each of the categories are listed in this article's Table E1 in the Online Repository at www.jacionline.org.
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Response to treatment. The bars represent response rates after 1 month of therapy (A) and reasons for drug discontinuation (B). Responses were defined as complete (normalization of absolute eosinophil

Response to treatment. The bars represent response rates after 1 month of therapy (A) and reasons for drug discontinuation (B). Responses were defined as complete (normalization of absolute eosinophil count [AEC] and clinical symptom improvement), partial (reduction of AEC, but not to normal levels, and/or improvement in symptoms), or no response (neither reduction of AEC nor improvement in symptoms).
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Association of serum TARC but not serum IgE levels with prednisone responsiveness. Serum IgE (A) and TARC (B) levels for prednisone responders (n = 129 and 75, respectively) and nonresponders (n = 13

Association of serum TARC but not serum IgE levels with prednisone responsiveness. Serum IgE (A) and TARC (B) levels for prednisone responders (n = 129 and 75, respectively) and nonresponders (n = 13 and 8, respectively) are shown by using box-and-whisker plots. The whiskers represent the minimum and maximum values and the horizontal lines represent the lower quartile, median, and upper quartile.
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Supported by the Division of Intramural Research of the NIAID/NIH (A.D.K., P.U.O., T.B.N.), grants AI41472 and AI72265 from the NIH (B.S.B.), grant AI061097 from the NIH (G.J.G.), the Human Immunology grant program of the Dana Foundation (B.S.B.), the Swiss National Science Foundation (H.-U.S.), the Belgian National Fund for Scientific Research (F.R.), and the Campaign Urging Research for Eosinophilic Disorders (M.E.R.). B.S.B. is a Cosner Scholar in Translational Research from Johns Hopkins University. The funding organizations had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; and preparation or review of the manuscript. The manuscript was approved by the Division of Intramural Research, NIAID/NIH.

Disclosure of potential conflict of interest: G. J. Gleich and K. M. Leiferman have equity in Ception, have received research support from GlaxoSmithKline, and are on the advisory board for APFE. J. E. Kahn has received consulting fees and research support from GlaxoSmithKline. T. B. Nutman is a stockholder in Johnson & Johnson and is employed by the National Institutes of Health. J. Ring has received research support from Novartis, Schering-Plough, Fujisawa, GlaxoSmithKline, Bencard, Stallergenes, ALK-Abelló, Allergopharma, Pharmacia, DPC Biermann, Aventis, Almirall, Leo, Galderma, and Switch Biotech. M. E. Rothenberg is a speaker and consultant for Merck; is a consultant for Ception Therapeutics, Novartis, Nycomed, and Centocor; has received research support from the National Institutes of Health, FAAN, and the Dana Foundation; is on the Medical Advisory Board for APFED; and is on the Executive Council for the International Eosinophil Society. F. Roufosse has received consulting fees from GlaxoSmithKline. J. Sheikh is on the speakers' bureau for Alcon, Meda, Sanofi-Aventis, and UBC; is a consultant for and is on the Advisory Board for Zeer.com; has received research support from GlaxoSmithKline; has been a legal consultant on the topics of allergy/immunology medical malpractice and latex allergy; is a member of the ACAAI; and is on the Executive Board of the Massachusetts Allergy Society (Secretary) and the New England Society of Allergy (CME Director). H.-U. Simon has received consulting fees from Pfizer, has received honoraria from Merck, and has received research support from the Swiss National Science Foundation, GlaxoSmithKline, and AstraZeneca. A. Wardlaw has received honoraria and research support from GlaxoSmithKline. P. F. Weller has served as a consultant for GlaxoSmithKline and has received research support from Merck. The rest of the authors have declared that they have no conflict of interest.

PII: S0091-6749(09)01410-9

doi: 10.1016/j.jaci.2009.09.022

« Previous Next » The Journal of Allergy and Clinical Immunology
Volume 124, Issue 6 , Pages 1319-1325.e3 , December 2009