Yan X, Li B, Qin T J, Qu S Q, Pan L J, Wu J Y, Liu D, Xiao Z J, Xu Z F
State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China.
Zhonghua Xue Ye Xue Za Zhi. 2021 Jan 14;42(1):15-20. doi: 10.3760/cma.j.issn.0253-2727.2021.01.004.
To evaluate the prognostic value of MIPSS70-plus in Chinese patients with primary myelofibrosis (PMF) . A total of 113 Chinese patients with PMF were retrospectively analyzed. The Kaplan-Meier method, Log-rank test, and Cox proportional hazard regression model were performed to evaluate the prognostic factors. The likelihood ratio test was used to evaluate the predictive power between MIPSS70-plus and DIPSS systems. The median age of the Chinese patients was 55 (range: 20-70) years, including 71 males and 42 females. According to the standard of MIPSS70-plus system, 99 patients (79.6% ) had a favorable karyotype and 23 patients (20.4% ) had an unfavorable karyotype. JAK2V617F in 55.8% (=63) , CALR exon9 in 17.7% (including 15 CALR type 1 and 5 CALR type 2, =20) , MPLW515 in 4.4% (=5) , and triple negative (no detectable JAK2, MPL, and CALR mutations) in 22.1% of patients in our cohort were found by target-specific next-generation sequencing approach. At least one high-molecular risk mutations were presented in 45.1% (=51) of patients, with ASXL1 in 38.9% (=44) , SRSF2 in 7.1% (=8) , IDH1/2 in 4.4% (=5) , and EZH2 in 3.5% (=4) of patients. A total of 28 patients (26.7% ) were in low risk, 20 (19.0% ) in intermediate risk, 41 (39.0% ) in high risk, and 16 (15.3% ) in very-high risk categories, which were delineated for the MIPSS70-plus model. A 2-year OS was 100% in low risk, 89.7% (95% 76.2% -100.0% ) in intermediate risk, 64.8% (95% 47.0% -82.6% ) in high risk, and 35.0% (95% 10.3% -59.7% ) in very-high risk categories, which had a significant difference (<0.001) . A significantly higher predictive power for survival of the MIPSS70-plus group was observed compared with the DIPSS group (=0.001, -2 log-likelihood ratios of 86.355 95.990 for the MIPSS70-plus and DIPSS systems, respectively) . The MIPSS70-plus had significantly higher predictive power than the DIPSS.
评估MIPSS70-plus在中国原发性骨髓纤维化（PMF）患者中的预后价值。对113例中国PMF患者进行回顾性分析。采用Kaplan-Meier法、Log-rank检验和Cox比例风险回归模型评估预后因素。采用似然比检验评估MIPSS70-plus与DIPSS系统之间的预测能力。中国患者的中位年龄为55岁（范围：20-70岁），其中男性71例，女性42例。根据MIPSS70-plus系统标准，99例患者（79.6%）核型良好，23例患者（20.4%）核型不良。通过靶向特异性二代测序方法发现，我们队列中55.8%（=63）的患者存在JAK2V617F突变，17.7%（包括15例CALR 1型和5例CALR 2型，=20）的患者存在CALR外显子9突变，4.4%（=5）的患者存在MPLW515突变，22.1%的患者为三阴性（未检测到JAK2、MPL和CALR突变）。45.1%（=51）的患者至少存在一种高分子风险突变，其中38.9%（=44）的患者存在ASXL1突变，7.1%（=8）的患者存在SRSF2突变，4.4%（=5）的患者存在IDH1/2突变，3.5%（=4）的患者存在EZH2突变。根据MIPSS70-plus模型划分，共有28例患者（26.7%）为低风险，20例（19.0%）为中风险，41例（39.0%）为高风险，16例（15.3%）为极高风险。低风险患者的2年总生存率为100%，中风险患者为89.7%（95% 76.2%-100.0%），高风险患者为64.8%（95% 47.0%-82.6%），极高风险患者为35.0%（95% 10.3%-59.7%），差异有统计学意义（<0.001）。与DIPSS组相比，MIPSS70-plus组对生存的预测能力显著更高（=0.001，MIPSS70-plus和DIPSS系统的-2对数似然比分别为86.355和95.990）。MIPSS70-plus的预测能力显著高于DIPSS。
