title: A dynamic prognostic model to predict survival in post-polycythemia vera myelofibrosis.
abstract: Post-polycythemia vera myelofibrosis (post-PV MF) is a late evolution of PV. In 647 patients with PV, we found that leukocytosis leukocyte count>(15x10(9)/L) at diagnosis is a risk factor for the evolution of post-PV MF. In a series of 68 patients who developed post-PV MF, median survival was 5.7 years. Hemoglobin level less than 100 g/L (10 g/dL) at diagnosis of post-PV MF was an independent risk factor for survival. The course of post-PV MF, however, is a dynamic process that implies a progressive worsening of clinical parameters. Using a multivariate Cox proportional hazard regression with time-dependent covariates, we found that a dynamic score based on hemoglobin level less than 100 g/L (10 g/dL), platelet count less than 100x10(9)/L, and leukocyte count more than 30x10(9)/L is useful to predict survival at any time from diagnosis of post-PV MF. The resulting hazard ratio of the score was 4.2 (95% CI: 2.4-7.7; P<.001), meaning a 4.2-fold worsening of survival for each risk factor acquired during follow up. In conclusion, leukocytosis at diagnosis of PV is a risk factor for evolution in post-PV MF. A dynamic score based on hemoglobin level, and platelet and leukocyte count predicts survival at any time from diagnosis of post-PV MF.
authors.affiliation: Department of Hematology, Fondazione Istituto di ricovero e cura a carattere scientifico Policlinico San Matteo, University of Pavia, Italy. f.passamonti@smatteo.pv.it
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.forename: Elisa
authors.initials: E
authors.lastname: Rumi
authors.forename: Marianna
authors.initials: M
authors.lastname: Caramella
authors.forename: Chiara
authors.initials: C
authors.lastname: Elena
authors.forename: Luca
authors.initials: L
authors.lastname: Arcaini
authors.forename: Emanuela
authors.initials: E
authors.lastname: Boveri
authors.forename: Cecilia
authors.initials: C
authors.lastname: Del Curto
authors.forename: Daniela
authors.initials: D
authors.lastname: Pietra
authors.forename: Laura
authors.initials: L
authors.lastname: Vanelli
authors.forename: Paolo
authors.initials: P
authors.lastname: Bernasconi
authors.forename: Cristiana
authors.initials: C
authors.lastname: Pascutto
authors.forename: Mario
authors.initials: M
authors.lastname: Cazzola
authors.forename: Enrica
authors.initials: E
authors.lastname: Morra
authors.forename: Mario
authors.initials: M
authors.lastname: Lazzarino
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005500:Follow-Up Studies; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007958:Leukocyte Count; D007964:Leukocytosis / Q000097:blood / Q000175:diagnosis / Q000401:mortality; D008297:Male; D008875:Middle Aged; D008954:Models, Biological; D010976:Platelet Count; D011087:Polycythemia Vera / Q000097:blood* / Q000150:complications / Q000175:diagnosis / Q000401:mortality*; D011237:Predictive Value of Tests; D055728:Primary Myelofibrosis / Q000097:blood* / Q000175:diagnosis / Q000209:etiology / Q000401:mortality*; D011379:Prognosis; D012044:Regression Analysis; D012307:Risk Factors; D015996:Survival Rate
chemical_list: D006454:Hemoglobins