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Blood, 26 March 2009, Vol. 113, No. 13, pp. 2895-2901. Prepublished online as a Blood First Edition Paper on November 6, 2008; DOI 10.1182/blood-2008-07-170449. This Article Full Text (PDF) Supplemental Table and Figures All Versions of this Article: blood-2008-07-170449v1 113/13/2895    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 Cervantes, F. Articles by Tefferi, A. Search for Related Content PubMed PubMed Citation Articles by Cervantes, F. Articles by Tefferi, A. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted July 23, 2008 Accepted October 21, 2008 A new prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment Francisco Cervantes*, Brigitte Dupriez, Arturo Pereira, Francesco Passamonti, John T. Reilly, Enrica Morra, Alessandro M Vannucchi, Ruben A Mesa, Jean-Loup Demory, Giovanni Barosi, Elisa Rumi, and Ayalew Tefferi Hematology Department, Hospital Clinic, IDIBAPS, University of Barcelona, Barcelona, Spain Centre Hospitalier, Lens and Lille, France Hematology Department, Fondazione IRCCS Policlinico S. Matteo, University of Pavia, Pavia, Italy Royal Hallamshire Hospital, Sheffield, United Kingdom University Milano-Niguarda, Milano, Italy University of Florence, Florence, Italy Division of Hematology, Mayo Clinic College of Medicine, Rochester, MN, United States Unit of Clinical Epidemiology, Center for the Study of Myelofibrosis, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy * Corresponding author; email: fcervan{at}clinic.ub.es. Therapeutic decision-making in primary myelofibrosis (PMF) is becoming more challenging because of the increasing use of allogeneic stem cell transplantation and new investigational drugs. In order to enhance this process by developing a highly discriminative prognostic system, 1,054 patients consecutively diagnosed with PMF at seven centers were studied. Overall median survival was 69 months (95% CI: 61-76). Multivariate analysis of parameters obtained at disease diagnosis identified age > 65 years, presence of constitutional symptoms, hemoglobin level < 10 g/dL, leukocyte count > 25 x 109/L and circulating blast cells 1% as predictors of shortened survival. Based on the presence of 0 (low risk), 1 (intermediate risk-1), 2 (intermediate risk-2) or 3 (high risk) of these variables, four risk groups with no overlapping in their survival curves were delineated; respective median survivals were 135, 95, 48 and 27 months (p<0.0001). Compared to prior prognostic models, the new risk stratification system displayed higher predictive accuracy, replicability and discriminating power. In 409 patients with assessable metaphases, cytogenetic abnormalities were associated with shorter survival but their independent contribution to prognosis was restricted to patients in the intermediate risk groups. JAK2V617F did not cluster with a specific risk group or affect survival. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: Y. Wang, W. Fiskus, D. G. Chong, K. M. Buckley, K. Natarajan, R. Rao, A. Joshi, R. Balusu, S. Koul, J. Chen, et al. Cotreatment with panobinostat and JAK2 inhibitor TG101209 attenuates JAK2V617F levels and signaling and exerts synergistic cytotoxic effects against human myeloproliferative neoplastic cells Blood, December 3, 2009; 114(24): 5024 - 5033. [Abstract] [Full Text] [PDF] R. Vaidya, S. Siragusa, J. Huang, S. M. Schwager, C. A. Hanson, K. Hussein, A. Pardanani, and A. Tefferi Mature Survival Data for 176 Patients Younger Than 60 Years With Primary Myelofibrosis Diagnosed Between 1976 and 2005: Evidence for Survival Gains in Recent Years Mayo Clin. Proc., December 1, 2009; 84(12): 1114 - 1119. [Abstract] [Full Text] [PDF] C. S. Tam, H. Kantarjian, J. Cortes, A. Lynn, S. Pierce, L. Zhou, M. J. Keating, D. A. Thomas, and S. 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