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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 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 Citation Map 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. Related Collections Phagocytes Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS 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 From the National Institute of Allergy and Infectious Diseases, National Heart, Lung, and Blood Institute, National Cancer Institute, National Eye Institute, National Institute of Neurologic Diseases and Stroke, and the Warren Grant Magnusson Clinical Center, National Institutes of Health, Bethesda, MD. 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 (NLs) underwent a detailed clinical evaluation at the National Institutes of Health (NIH). No clinical abnormalities were more frequent in the family members with FE compared with the NLs. There was, however, a decreased prevalence of asthma in family members with FE compared with 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 eosinophil-derived neurotoxin (EDN) and major basic protein (MBP) were elevated in patients with FE compared with NL, levels of both granule proteins were lower than in nonfamilial 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 compared with 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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