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Blood, 4 June 2009, Vol. 113, No. 23, pp. 5727-5736. Prepublished online as a Blood First Edition Paper on April 10, 2009; DOI 10.1182/blood-2009-02-205237. This Article Full Text (PDF) All Versions of this Article: blood-2009-02-205237v1 113/23/5727    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 Lim, K.-H. Articles by Pardanani, A. Search for Related Content PubMed PubMed Citation Articles by Lim, K.-H. Articles by Pardanani, A. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted February 12, 2009 Accepted March 24, 2009 Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors Ken-Hong Lim, Ayalew Tefferi, Terra L. Lasho, Christy Finke, Mrinal Patnaik, Joseph H. Butterfield, Rebecca F. McClure, Chin-Yang Li, and Animesh Pardanani* Division of Hematology, Mayo Clinic, Rochester, MN, United States Division of Allergic Diseases, Mayo Clinic, Rochester, MN, United States Division of Hematopathology, Mayo Clinic, Rochester, MN, United States * Corresponding author; email: pardanani.animesh{at}mayo.edu. Clinical phenotype in systemic mastocytosis (SM) is markedly variable, which complicates prognostication and decision making regarding the choice and timing of therapy. In a retrospective study of 342 consecutive adult patients with SM seen at the Mayo Clinic between 1976 and 2007, disease sub-designation according to the World Health Organization (WHO) proposal was indolent (ISM) in 159 (46%), with associated clonal hematological non-mast cell lineage disease (SM-AHNMD) in 138 (40%), aggressive (ASM) in 41 (12%) and mast cell leukemia in 4 (1%). KITD816V was detected in bone marrow-derived DNA by allele-specific PCR in 68% of 165 patients evaluated (ISM 78%, ASM 82%, SM-AHNMD 60%; p=0.03); JAK2V617F was detected in 4%, all in SM-AHNMD. Compared to those with non-indolent SM, life-expectancy in ISM was superior and not significantly different than that of the age- and gender-matched U.S. population. In addition, multivariable analysis identified advanced age, weight loss, anemia, thrombocytopenia, hypoalbuminemia, and excess bone marrow blasts as independent adverse prognostic factors for survival. The current study validates the prognostic relevance of the WHO subclassification of SM and provides additional information of value in terms of both risk stratification and interpretation of clinical presentation and laboratory results. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A. Pardanani, K.-H. Lim, T. L. Lasho, C. Finke, R. F. McClure, C.-Y. Li, and A. Tefferi Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignancies Blood, October 29, 2009; 114(18): 3769 - 3772. [Abstract] [Full Text] [PDF]

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