title: DIPSS plus: a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status.
abstract: OBJECTIVE
  The Dynamic International Prognostic Scoring System (DIPSS) for primary myelofibrosis (PMF) uses five risk factors to predict survival: age older than 65 years, hemoglobin lower than 10 g/dL, leukocytes higher than 25 × 10(9)/L, circulating blasts ≥ 1%, and constitutional symptoms. The main objective of this study was to refine DIPSS by incorporating prognostic information from karyotype, platelet count, and transfusion status.
  
  
  METHODS
  Mayo Clinic databases for PMF were used to identify patients with available bone marrow histologic and cytogenetic information.
  
  
  RESULTS
  Seven hundred ninety-three consecutive patients were selected and divided into two groups based on whether or not their referral occurred within (n = 428; training set) or after (n = 365; test set) 1 year of diagnosis. Multivariable analysis identified DIPSS, unfavorable karyotype, platelets lower than 100 × 10(9)/L, and transfusion need as independent predictors of inferior survival. Hazard ratio (HR)-weighted adverse points were assigned to these variables to develop a composite prognostic model using the training set. The model was subsequently validated in the test set, and its application to all 793 patients resulted in median survivals of 185, 78, 35, and 16 months for low, intermediate-1 (HR, 2.2; 95% CI, 1.4 to 3.6), intermediate-2 (HR, 4.9; 95% CI, 3.2 to 7.7), and high-risk groups (HR, 10.7; 95% CI, 6.8 to 16.9), respectively (P < .001). Leukemia-free survival was predicted by the presence of thrombocytopenia or unfavorable karyotype (10-year risk of 31% v 12%; HR, 3.3; 95% CI, 1.9 to 5.6).
  
  
  CONCLUSIONS
  DIPSS plus effectively combines prognostic information from DIPSS, karyotype, platelet count, and transfusion status to predict overall survival in PMF. In addition, unfavorable karyotype or thrombocytopenia predicts inferior leukemia-free survival.
authors.affiliation: Mayo Clinic, Rochester, MN 55905, USA.
authors.forename: Naseema
authors.initials: N
authors.lastname: Gangat
authors.forename: Domenica
authors.initials: D
authors.lastname: Caramazza
authors.forename: Rakhee
authors.initials: R
authors.lastname: Vaidya
authors.forename: Geeta
authors.initials: G
authors.lastname: George
authors.forename: Kebede
authors.initials: K
authors.lastname: Begna
authors.forename: Susan
authors.initials: S
authors.lastname: Schwager
authors.forename: Daniel
authors.initials: D
authors.lastname: Van Dyke
authors.forename: Curtis
authors.initials: C
authors.lastname: Hanson
authors.forename: Wenting
authors.initials: W
authors.lastname: Wu
authors.forename: Animesh
authors.initials: A
authors.lastname: Pardanani
authors.forename: Francisco
authors.initials: F
authors.lastname: Cervantes
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001803:Blood Transfusion / Q000401:mortality*; D001856:Bone Marrow Examination; D002471:Cell Transformation, Neoplastic / Q000235:genetics; D016009:Chi-Square Distribution; D019992:Databases as Topic; D018572:Disease-Free Survival; D005260:Female; D006305:Health Status Indicators; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D053208:Kaplan-Meier Estimate; D007621:Karyotyping; D007938:Leukemia / Q000097:blood / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality* / Q000628:therapy; D007958:Leukocyte Count; D008297:Male; D008875:Middle Aged; D010976:Platelet Count; D011237:Predictive Value of Tests; D055728:Primary Myelofibrosis / Q000097:blood / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality* / Q000628:therapy; D011379:Prognosis; D016016:Proportional Hazards Models; D015203:Reproducibility of Results; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors; D055815:Young Adult
chemical_list: D006454:Hemoglobins