title: Improving survival trends in primary myelofibrosis: an international study.
abstract: OBJECTIVE
  Despite the lack of major improvements in the treatment of primary myelofibrosis (PMF), there are recent indications that the survival of patients might have increased over the years. This study was aimed at ascertaining whether survival prolongation has actually occurred in PMF.
  
  
  METHODS
  A total of 802 patients diagnosed with PMF in four European countries were compared for the presentation of features and survival according to the diagnostic periods 1980 to 1995 (n = 434) and 1996 to 2007 (n = 368); relative survival was estimated for the two groups.
  
  
  RESULTS
  Patients diagnosed between 1996 and 2007 more often had constitutional symptoms (31% v 23%) but a lower incidence of marked anemia (31% v 39%), leukocytosis greater than 25 × 10(9)/L (9% v 13%), and blood blasts (27% v 33%); risk distribution was comparable between the two groups. Median survival was 4.6 years (95% CI, 4.0 to 5.1) for patients from 1980 to 1995 and 6.5 years (95% CI, 5.5 to 7.4) for patients from 1996 to 2007 (P < .001). The latter group of patients showed improved relative survival, especially for women, patients younger than age 65 years, and patients with low or intermediate-1-risk disease. Rates of PMF-attributable mortality at 5 and 10 years were significantly lower in the second period; this reduction in disease-specific mortality occurred across all patient subgroups, except in intermediate-2-risk or high-risk patients.
  
  
  CONCLUSIONS
  Survival of PMF is steadily improving, except in patients in poor-risk categories. This observation must be taken into account at the time of evaluating the survival impact of newer therapies for PMF, which are currently being tested in these patient subpopulations.
authors: Hospital Clínic, Institut d’Investigació Biomèdica August Pi i Sunyer, University of Barcelona, Spain. fcervan@clinic.ub.es
authors: Francisco
authors: F
authors: Cervantes
authors: Brigitte
authors: B
authors: Dupriez
authors: Francesco
authors: F
authors: Passamonti
authors: Alessandro M
authors: AM
authors: Vannucchi
authors: Enrica
authors: E
authors: Morra
authors: John T
authors: JT
authors: Reilly
authors: Jean-Loup
authors: JL
authors: Demory
authors: Elisa
authors: E
authors: Rumi
authors: Paola
authors: P
authors: Guglielmelli
authors: Elisa
authors: E
authors: Roncoroni
authors: Ayalew
authors: A
authors: Tefferi
authors: Arturo
authors: A
authors: Pereira
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D005060:Europe; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy; D012306:Risk; D012737:Sex Factors; D015996:Survival Rate / Q000639:trends; D055815:Young Adult