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Blood, 1 October 2007, Vol. 110, No. 7, pp. 2331-2333. Prepublished online as a Blood First Edition Paper on July 16, 2007; DOI 10.1182/blood-2006-06-028100. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2006-06-028100v1 110/7/2331    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 CrossRef Citing Articles via Google Scholar Google Scholar Articles by Akin, C. Articles by Metcalfe, D. D. Search for Related Content PubMed PubMed Citation Articles by Akin, C. Articles by Metcalfe, D. D. Related Collections Stem Cells in Hematology Brief Reports Clinical Trials and Observations Immunobiology Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Brief Report Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with "idiopathic" anaphylaxis Cem Akin1,2, Linda M. Scott1, Can N. Kocabas1, Nataliya Kushnir-Sukhov1, Erica Brittain3, Pierre Noel4, and Dean D. Metcalfe1 1 Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health (NIH), Bethesda, MD; 2 Division of Allergy and Immunology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI; 3 Biostatistics Research Branch, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD; 4 The Clinical Center, NIH, Bethesda, MD Idiopathic anaphylaxis remains a perplexing disorder in which existing prophylactic therapy is inadequate. In this prospective study, we sought to determine whether patients with idiopathic anaphylaxis might have evidence for a clonal disorder of mast cells related to mastocytosis and for which novel targeted therapies might be considered. We report 12 patients with "idiopathic" anaphylaxis who did not exhibit either urticaria pigmentosa or the characteristic bone marrow biopsy finding of multifocal mast-cell aggregates observed in systemic mastocytosis. Of these 12 patients, 5 had evidence of 1 or more minor criteria for mastocytosis. C-KIT mutational analysis was positive for the 816D>V activating mutation in 3 of 3 patients in CD25+ bone marrow cells where the analysis was performed. These results demonstrate the presence of an aberrant mast-cell population carrying clonal markers in a subset of patients diagnosed with "idiopathic" anaphylaxis, who may respond to inhibitors targeting mutated C-KIT. This intramural clinical trial was conducted in 2003 and 2004 and was registered at http://clinicalcenter.nih.gov with a study number 03-I-0010. Since the study is now closed, it is no longer available online. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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