Hemocentro, Centro de Hematologia e Hemoterapia, Universidade Estadual de Campinas, Campinas, São Paulo, Brazil.
Clinics (Sao Paulo). 2013;68(3):339-43. doi: 10.6061/clinics/2013(03)oa09.
To evaluate whether risk scores used to classify patients with primary myelofibrosis and JAK-2 V617F mutation status can predict clinical outcome.
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.
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).
These results emphasize the importance of the IPSS prognostic score for risk assessment in predicting the clinical outcome of primary myelofibrosis patients.
评估用于分类原发性骨髓纤维化患者和 JAK-2 V617F 突变状态的风险评分是否可以预测临床结局。
回顾分析了 1992 年至 2011 年间诊断的 74 例原发性骨髓纤维化患者的临床和实验室数据。计算了 IPSS 和 Lille 评分进行风险分层，并与总生存期相关联。
32 例（47%）检测到 JAK2 V617F 突变，但与总生存期无显著相关性。根据评分对患者进行分类：Lille-低，53 例（73%）；中，13 例（18%）；高，5 例（7%）；IPSS-低，15 例（26%）；中-1，23 例（32%）；中-2，19 例（26%）；高，15 例（31%）。根据 IPSS（高和中-2）风险较高的患者总生存期明显短于低风险组（中-1 和低）（p = 0.02）。
这些结果强调了 IPSS 预后评分在预测原发性骨髓纤维化患者临床结局中的重要性。
