title: Validation and comparison of contemporary prognostic models in primary myelofibrosis: analysis based on 334 patients from a single institution.
abstract: BACKGROUND
  Survival in primary myelofibrosis (PMF) is predicted by several prognostic scoring systems (PSSs); the most widely recognized is that of Dupriez. Two other PSSs, Cervantes and Mayo, were recently reported as being more useful in younger patients. The current study compares these 3 PSSs among all age groups.
  
  
  METHODS
  The Mayo Clinic PMF database was queried to identify a consecutive series of patients in whom pretreatment bone marrow and complete blood count (CBC), obtained within 6 months of diagnosis, were available for review.
  
  
  RESULTS
  Among 334 study patients (median age, 57 years), median survival was 70 months. Multivariable analysis of all 6 adverse prognostic factors utilized in the aforementioned PSSs (ie, hemoglobin <10 g/dL, leukocyte count <4 or >30 x 10(9)/L, constitutional symptoms, circulating blasts > or = 1%, platelet count <100 x 10(9)/L, absolute monocyte count > or = 1 x 10(9)/L) identified all but platelet count as being significant. The Mayo PSS, which is based on the 4 CBC-derived parameters (ie, hemoglobin, platelet, leukocyte, and monocyte counts), displayed a better hazard ratio profile compared with the other 2 PSSs in identifying long-lived patients as well as delineating intermediate-risk disease category. The latter effect was even more pronounced in patients younger than age 60 years.
  
  
  CONCLUSIONS
  The Mayo PSS for survival in PMF is an objective CBC-derived prognostic model that might be superior in its performance over that of either the Dupriez and Cervantes PSSs.
authors.affiliation: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA. tefferi.ayalew@mayo.edu
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
authors.forename: Jocelin
authors.initials: J
authors.lastname: Huang
authors.forename: Susan
authors.initials: S
authors.lastname: Schwager
authors.forename: Chin-Yang
authors.initials: CY
authors.lastname: Li
authors.forename: Wenting
authors.initials: W
authors.lastname: Wu
authors.forename: Animesh
authors.initials: A
authors.lastname: Pardanani
authors.forename: Ruben A
authors.initials: RA
authors.lastname: Mesa
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001772:Blood Cell Count; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D015233:Models, Statistical; D055728:Primary Myelofibrosis / Q000097:blood / Q000401:mortality*; D011379:Prognosis; D012307:Risk Factors; D016019:Survival Analysis; D015996:Survival Rate
chemical_list: D006454:Hemoglobins