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Prepublished online as a Blood First Edition Paper on April 3, 2003; DOI 10.1182/blood-2003-01-0006. This Article Full Text (PDF) All Versions of this Article: 2003-01-0006v1 101/12/4660    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 Related Collections Related Article in Blood Online Social Bookmarking                   What's this? Submitted January 2, 2003 Accepted March 25, 2003 Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness Amy D Klion*, Pierre Noel, Cem Akin, Melissa A Law, D G Gilliland, Jan Cools, Dean D Metcalfe, and Thomas B Nutman Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA Department of Laboratory Medicine, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD, USA Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA * Corresponding author; email: aklion{at}nih.gov. Since serum tryptase levels are elevated in some patients with myeloproliferative disorders, we examined their utility in identifying a subset of patients with hypereosinophilic syndrome (HES) and an underlying myeloproliferative disorder. Elevated serum tryptase levels (>11.5 ng/ml) were present in 9/15 patients with HES and were associated with other markers of myeloproliferation, including elevated B12 levels and splenomegaly. Although bone marrow biopsies in these patients showed increased numbers of CD25+ mast cells and atypical spindle-shaped mast cells, patients with HES and elevated serum tryptase could be distinguished from patients with systemic mastocytosis and eosinophilia by their clinical manifestations, the absence of mast cell aggregates, the lack of a somatic KIT mutation and the presence of the recently described FIP1L1-PDGFRA fusion. Patients with HES and elevated serum tryptase were more likely to develop fibroproliferative end organ damage, and 3/9 died within 5 years of diagnosis in contrast to 0/6 patients with normal serum tryptase levels. All 6 patients with HES and elevated tryptase treated with imatinib demonstrated a clinical and hematologic response. In summary, elevated serum tryptase appears to be a sensitive marker of a myeloproliferative variant of HES that is characterized by tissue fibrosis, poor prognosis, and imatinib-responsiveness. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Major link between mast cells and the idiopathic hypereosinophilic syndrome Marc E. Rothenberg Blood 2003 101: 4647-4648. [Full Text] [PDF] This article has been cited by other articles: A. D. Klion How I treat hypereosinophilic syndromes Blood, October 29, 2009; 114(18): 3736 - 3741. [Abstract] [Full Text] [PDF] F. 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