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Blood, 1 June 2004, Vol. 103, No. 11, pp. 4050-4055. Prepublished online as a Blood First Edition Paper on February 26, 2004; DOI 10.1182/blood-2003-11-3850. This Article Full Text (PDF) All Versions of this Article: 2003-11-3850v1 103/11/4050    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Klion, A. D Articles by Nutman, T. B Search for Related Content PubMed PubMed Citation Articles by Klion, A. D Articles by Nutman, T. B Social Bookmarking                   What's this? Submitted November 13, 2003 Accepted February 11, 2004 Familial eosinophilia: a benign disorder? Amy D Klion*, Melissa A Law, William Riemenschneider, Mary Lou McMaster, Margaret R Brown, McDonald Horne, Barbara Karp, Michael Robinson, Vandana Sachdev, Eben Tucker, Maria Turner, and Thomas B Nutman National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, USA National Cancer Institute, National Institutes of Health, Bethesda, MD, USA Warren Grant Magnusson Clinical Center, National Institutes of Health, Bethesda, MD, USA National Institute of Neurologic Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA National Eye Institute, National Institutes of Health, Bethesda, MD, USA * Corresponding author; email: aklion{at}niaid.nih.gov. Familial eosinophilia (FE) is an autosomal dominant disorder characterized by marked eosinophilia and progression to end organ damage in some, but not all, affected family members. To better define the pathogenesis of FE, 13 affected and 11 unaffected family members (NL) underwent a detailed clinical evaluation at the NIH. No clinical abnormalities were more frequent in the family members with FE. There was, however, a decreased prevalence of asthma in family members with FE as compared to unaffected family members. Eosinophil morphology as assessed by either light or transmission electron microscopy, was normal in family members with and without FE. Although levels of EDN and MBP were elevated in patients with FE as compared to NL, levels of both granule proteins were lower than in non-familial hypereosinophilic syndrome (HES). Similarly, increased surface expression of the activation markers CD69, CD25 and HLA-DR was detected by flow cytometry on eosinophils from patients with FE as compared to NL, albeit less than that seen in HES. These data suggest that, despite prolonged marked eosinophilia, FE can be distinguished from HES by a more benign clinical course that may be related to a relative lack of eosinophil activation. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: H. H. Norris, M. E. Peterson, C. C. Stebbins, B. W. McConchie, V. G. Bundoc, S. Trivedi, M. G. Hodges, R. M. Anthony, J. F. Urban Jr, E. O. Long, et al. Inhibitory receptor gp49B regulates eosinophil infiltration during allergic inflammation J. Leukoc. Biol., December 1, 2007; 82(6): 1531 - 1541. [Abstract] [Full Text] [PDF] A. Tefferi Blood Eosinophilia: A New Paradigm in Disease Classification, Diagnosis, and Treatment Mayo Clin. Proc., January 1, 2005; 80(1): 75 - 83. [Abstract] [PDF]

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