Primary myelofibrosis: risk stratification by IPSS identifies patients with poor clinical outcome

• Main Authors: Bruno Deltreggia Benites, Carolina Silva Costa Lima, Irene Lorand-Metze, Marcia Torresan Delamain, Gislaine Borba Oliveira, Daiane de Almeida, Carmino Antonio de Souza, Jose Vassallo, Katia Borgia Barbosa Pagnano
• Format: Article
• Language: English
• Published: Faculdade de Medicina / USP 2013-01-01
• Series: Clinics
• Subjects: Primary Myelofibrosis; IPSS; Prognosis
• Online Access: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322013000300010

OBJECTIVES: 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.