title: A dynamic prognostic model to predict survival in primary myelofibrosis: a study by the IWG-MRT (International Working Group for Myeloproliferative Neoplasms Research and Treatment).
abstract: Age older than 65 years, hemoglobin level lower than 100 g/L (10 g/dL), white blood cell count greater than 25 x 10(9)/L, peripheral blood blasts 1% or higher, and constitutional symptoms have been shown to predict poor survival in primary myelofibrosis (PMF) at diagnosis. To investigate whether the acquisition of these factors during follow-up predicts survival, we studied 525 PMF patients regularly followed. All 5 variables had a significant impact on survival when analyzed as time-dependent covariates in a multivariate Cox proportional hazard model and were included in 2 separate models, 1 for all patients (Dynamic International Prognostic Scoring System [DIPSS]) and 1 for patients younger than 65 years (age-adjusted DIPSS). Risk factors were assigned score values based on hazard ratios (HRs). Risk categories were low, intermediate-1, intermediate-2, and high in both models. Survival was estimated by the HR. When shifting to the next risk category, the HR was 4.13 for low risk, 4.61 for intermediate-1, and 2.54 for intermediate-2 according to DIPSS; 3.97 for low risk, 2.84 for intermediate-1, and 1.81 for intermediate-2 according to the age-adjusted DIPSS. The novelty of these models is the prognostic assessment of patients with PMF anytime during their clinical course, which may be useful for treatment decision-making.
authors.affiliation: Department of Hematology Oncology, University of Pavia & Fondazione, Istituto Di Ricovero e Cura a Carattere Scientifico, Policlinico San Matteo, Pavia, Italy. f.passamonti@smatteo.pv.it
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.forename: Francisco
authors.initials: F
authors.lastname: Cervantes
authors.forename: Alessandro Maria
authors.initials: AM
authors.lastname: Vannucchi
authors.forename: Enrica
authors.initials: E
authors.lastname: Morra
authors.forename: Elisa
authors.initials: E
authors.lastname: Rumi
authors.forename: Arturo
authors.initials: A
authors.lastname: Pereira
authors.forename: Paola
authors.initials: P
authors.lastname: Guglielmelli
authors.forename: Ester
authors.initials: E
authors.lastname: Pungolino
authors.forename: Marianna
authors.initials: M
authors.lastname: Caramella
authors.forename: Margherita
authors.initials: M
authors.lastname: Maffioli
authors.forename: Cristiana
authors.initials: C
authors.lastname: Pascutto
authors.forename: Mario
authors.initials: M
authors.lastname: Lazzarino
authors.forename: Mario
authors.initials: M
authors.lastname: Cazzola
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
mesh_terms: D000367:Age Factors; D000368:Aged; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D007958:Leukocyte Count; D008297:Male; D008875:Middle Aged; D008954:Models, Biological; D015233:Models, Statistical; D055728:Primary Myelofibrosis / Q000097:blood / Q000401:mortality* / Q000628:therapy; D011379:Prognosis; D016016:Proportional Hazards Models; D012307:Risk Factors
chemical_list: D006454:Hemoglobins