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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 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 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 Google Scholar Articles by Lim, K.-H. Articles by Pardanani, A. PubMed PubMed Citation Articles by Lim, K.-H. Articles by Pardanani, A. Related Collections Free Research Articles Myeloid Neoplasia Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors Ken-Hong Lim1,2, Ayalew Tefferi1, Terra L. Lasho1, Christy Finke1, Mrinal Patnaik1, Joseph H. Butterfield3, Rebecca F. McClure4, Chin-Yang Li4, and Animesh Pardanani1 1 Division of Hematology, Mayo Clinic, Rochester, MN; 2 Division of Hematology-Oncology, Mackay Memorial Hospital, and Mackay Medicine, Nursing and Management College, Taipei, Taiwan; and Divisions of 3 Allergic Diseases and 4 Hematopathology, Mayo Clinic, Rochester, MN 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 subdesignation according to the World Health Organization (WHO) proposal was indolent (ISM) in 159 (46%), with associated clonal hematologic 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 polymerase chain reaction (PCR) in 68% of 165 patients evaluated (ISM, 78%; ASM, 82%; SM-AHNMD, 60%; P = .03); JAK2V617F was detected in 4%, all in SM-AHNMD. Compared with those with nonindolent SM, life expectancy in ISM was superior and not significantly different from that of the age- and sex-matched US 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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