title: Red blood cell transfusion-dependency implies a poor survival in primary myelofibrosis irrespective of IPSS and DIPSS.
abstract: Risk stratification in primary myelofibrosis is currently based on two international prognostic scoring systems, neither of which takes into consideration red blood cell transfusion-dependency. In 288 consecutive patients with primary myelofibrosis, red blood cell transfusion-dependency at diagnosis affects survival independently of the International Prognostic Scoring System (P < 0.001). To evaluate the dynamic impact on survival of red blood cell transfusion-dependency, we performed a Cox's regression analysis with transfusion status as time-dependent covariate in 220 regularly followed patients with primary myelofibrosis. Patients who begin red blood cell transfusions anytime (n = 80, 36%) have a significantly worse survival compared to those who continue follow up without transfusions (HR: 7.8, 95%CI: 5.1-11.9; P < 0.001). Adjusting for Dynamic International Prognostic Scoring System in a multivariate analysis, red blood cell transfusion-dependency retained an independent prognostic impact on survival. This study suggests that red blood cell transfusion-dependency should be considered to improve risk stratification of primary myelofibrosis during follow up.
authors.affiliation: Department of Hematology Oncology, Division of Hematology, University of Pavia Medical School and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Elena
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.forename: Elisa
authors.initials: E
authors.lastname: Rumi
authors.forename: Luca
authors.initials: L
authors.lastname: Malcovati
authors.forename: Luca
authors.initials: L
authors.lastname: Arcaini
authors.forename: Emanuela
authors.initials: E
authors.lastname: Boveri
authors.forename: Michele
authors.initials: M
authors.lastname: Merli
authors.forename: Daniela
authors.initials: D
authors.lastname: Pietra
authors.forename: Cristiana
authors.initials: C
authors.lastname: Pascutto
authors.forename: Mario
authors.initials: M
authors.lastname: Lazzarino
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D017707:Erythrocyte Transfusion / Q000401:mortality*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate