Mechanisms of allergy and clinical immunologyHypereosinophilic syndrome: A multicenter, retrospective analysis of clinical characteristics and response to therapy
Section snippets
Methods
Patients meeting diagnostic criteria for HES,6 evaluated between January 2001 and December 2006 at 11 participating institutions with expertise in the evaluation of eosinophilic disorders, were included in the study. Inclusion criteria were documentation of a peripheral eosinophil count of ≥1.5 × 109/L and signs or symptoms of end organ involvement for which another etiology could not be found. Patients with neoplasms (other than Fip1-like 1–platelet-derived growth factor receptor α
Baseline characteristics
Of the 188 patients evaluated, 104 were male (55%), and 84 were female (45%). The median age at diagnosis was 45 years of age (range, 6-85 years). The peak recorded absolute eosinophil counts ranged from 1.5 to 400 × 109/L with a geometric mean (GM) peak eosinophil count of 6.6 × 109/L (Table I). Of the 161 patients tested for the FP mutation, 18 (11%), all of whom were male, were positive. Of the 168 patients who were evaluated for clonal or aberrant populations of T cells, 29 (17%; 15 male
Discussion
One of the major problems with previously reported series of patients with HES has been referral bias, with single centers more likely to see patients with end organ manifestations that fall within the area of expertise of a particular subspecialty interested in HES. This has been compounded by the tendency to publish cases of the most severely affected patients, a disproportionate number of whom, in retrospect, were likely FP-positive. Patients in the current series were referred to groups
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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.