New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment
New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment
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DOI：
10.1182/blood-2008-07-170449
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发表时间：
2009-03-26
期刊：
影响因子：
20.3
通讯作者：
Tefferi, Ayalew
中科院分区：
文献类型：
--
作者：
Cervantes, Francisco;Dupriez, Brigitte;Tefferi, Ayalew
由于异基因干细胞移植和新的研究药物的使用越来越多，原发性骨髓纤维化（PMF）的治疗决策变得越来越具有挑战性。为了加强这一进程的发展高度判别预后系统，1054例连续诊断PMF在7个中心进行了研究。总体中位生存期为69个月（95%置信区间[CI]：61 - 76）。对疾病诊断时获得的参数进行多变量分析，发现年龄大于65岁、存在全身症状、血红蛋白水平低于10 g/dL、白细胞计数大于25 × 10（9）/L、循环原始细胞1%或更高是生存期缩短的预测因素。根据这些变量中存在0（低风险）、1（中等风险-1）、2（中等风险-2）或大于或等于3（高风险），划定了4个风险组，其生存曲线没有重叠;各自的中位生存期为135、95、48和27个月（P <.001）。与以前的预后模型相比，新的危险分层系统显示出更高的预测准确性，可重复性和区分能力。在409例具有可评估中期分裂相的患者中，细胞遗传学异常与较短的生存期相关，但其对预后的独立贡献仅限于中危组患者。JAK2V617F不与特定风险组聚集或影响生存。（血。2009; 113：2895 - 2901）
Therapeutic decision-making in primary myelofibrosis (PMF) is becoming more challenging because of the increasing use of allogeneic stem cell transplantation and new investigational drugs. To enhance this process by developing a highly discriminative prognostic system, 1054 patients consecutively diagnosed with PMF at 7 centers were studied. Overall median survival was 69 months (95% confidence interval [CI]: 61-76). Multivariate analysis of parameters obtained at disease diagnosis identified age greater than 65 years, presence of constitutional symptoms, hemoglobin level less than 10 g/dL, leukocyte count greater than 25 x 10(9)/L, and circulating blast cells 1% or greater as predictors of shortened survival. Based on the presence of 0 (low risk), 1 (intermediate risk-1), 2 (intermediate risk-2) or greater than or equal to 3 (high risk) of these variables, 4 risk groups with no overlapping in their survival curves were delineated; respective median survivals were 135, 95, 48, and 27 months (P < .001). Compared with prior prognostic models, the new risk stratification system displayed higher predictive accuracy, replicability, and discriminating power. In 409 patients with assessable metaphases, cytogenetic abnormalities were associated with shorter survival, but their independent contribution to prognosis was restricted to patients in the intermediate-risk groups. JAK2V617F did not cluster with a specific risk group or affect survival. (Blood. 2009; 113: 2895-2901)