title: Primary myelofibrosis: risk stratification by IPSS identifies patients with poor clinical outcome.
abstract: OBJECTIVE
  To evaluate whether risk scores used to classify patients with primary myelofibrosis and JAK-2 V617F mutation status can predict clinical outcome.
  
  
  METHODS
  A review of clinical and laboratory data from 74 patients with primary myelofibrosis diagnosed between 1992 and 2011. The IPSS and Lille scores were calculated for risk stratification and correlated with overall survival.
  
  
  RESULTS
  A V617F JAK2 mutation was detected in 32 cases (47%), with no significant correlation with overall survival. The patients were classified according to the scores: Lille - low, 53 (73.%); intermediate, 13 (18%); and high, 5 (7%); and IPSS- low, 15 (26%); intermediate-1, 23 (32%); intermediate-2, 19 (26%); and high, 15 (31%). Those patients presenting a higher risk according to the IPSS (high and intermediate-2) had a significantly shorter overall survival relative to the low risk groups (intermediate-1 and low) (p = 0.02).
  
  
  CONCLUSIONS
  These results emphasize the importance of the IPSS prognostic score for risk assessment in predicting the clinical outcome of primary myelofibrosis patients.
authors: Hemocentro, Centro de Hematologia e Hemoterapia, Universidade Estadual de Campinas, Campinas, São Paulo, Brazil.
authors: Bruno Deltreggia
authors: BD
authors: Benites
authors: Carolina Silva Costa
authors: CS
authors: Lima
authors: Irene
authors: I
authors: Lorand-Metze
authors: Marcia Torresan
authors: MT
authors: Delamain
authors: Gislaine Borba
authors: GB
authors: Oliveira
authors: Daiane de
authors: Dd
authors: Almeida
authors: Carmino Antonio de
authors: CA
authors: Souza
authors: Jose
authors: J
authors: Vassallo
authors: Katia Borgia Barbosa
authors: KB
authors: Pagnano
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy; D011379:Prognosis; D015203:Reproducibility of Results; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D017211:Treatment Failure
chemical_list: C507924:JAK2 protein, human; D053614:Janus Kinase 2
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