title: New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment.
abstract: 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. To enhance this process by developing a highly discriminative prognostic system, 1054 patients consecutively diagnosed with PMF at 7 centers were studied. Overall median survival was 69 months (95% confidence interval [CI]: 61-76). Multivariate analysis of parameters obtained at disease diagnosis identified age greater than 65 years, presence of constitutional symptoms, hemoglobin level less than 10 g/dL, leukocyte count greater than 25 x 10(9)/L, and circulating blast cells 1% or greater as predictors of shortened survival. Based on the presence of 0 (low risk), 1 (intermediate risk-1), 2 (intermediate risk-2) or greater than or equal to 3 (high risk) of these variables, 4 risk groups with no overlapping in their survival curves were delineated; respective median survivals were 135, 95, 48, and 27 months (P< .001). Compared with 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.
authors.affiliation: Hematology Department, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain. fcervan@clinic.ub.es
authors.forename: Francisco
authors.initials: F
authors.lastname: Cervantes
authors.forename: Brigitte
authors.initials: B
authors.lastname: Dupriez
authors.forename: Arturo
authors.initials: A
authors.lastname: Pereira
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.forename: John T
authors.initials: JT
authors.lastname: Reilly
authors.forename: Enrica
authors.initials: E
authors.lastname: Morra
authors.forename: Alessandro M
authors.initials: AM
authors.lastname: Vannucchi
authors.forename: Ruben A
authors.initials: RA
authors.lastname: Mesa
authors.forename: Jean-Loup
authors.initials: JL
authors.lastname: Demory
authors.forename: Giovanni
authors.initials: G
authors.lastname: Barosi
authors.forename: Elisa
authors.initials: E
authors.lastname: Rumi
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002423:Cause of Death; D002648:Child; D003226:Congresses as Topic; D005260:Female; D006801:Humans; D007391:International Cooperation; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D012107:Research Design; D016019:Survival Analysis; D055815:Young Adult