MIPSS70-plus预后积分系统评估中国原发性骨髓纤维化患者预后的评价

Evaluation of the prognostic value of MIPSS70-plus in Chinese patients with primary myelofibrosis

目的评价MIPSS70-plus预后积分系统对中国原发性骨髓纤维化(PMF)患者的预后评估价值。方法回顾性分析113例PMF患者的临床资料,应用Log-rank和COX回归模型进行预后相关因素分析;应用似然比检验比较MIPSS70-plus和动态国际预后积分系统(DIPSS)的预后评估效能。结果 113例PMF患者中男71例,女42例,中位年龄55(20~70)岁。依据MIPSS70-plus染色体核型分组标准,染色体核型预后良好组90例(79.6%),预后不良组23例(20.4%)。二代测序基因突变检测结果示,JAK2V617F突变63例(55.8%),CALR外显子9突变20例(17.7%)(其中1型CALR突变15例,2型CALR突变5例),MPLW515突变5例(4.4%),25例(22.1%)未检测到JAK2、MPL和CALR基因突变(三阴性)。高分子风险(HMR)突变检出率依次为ASXL1突变44例(38.9%)、SRSF2突变8例(7.1%)、IDH1/2突变5例(4.4%)、EZH2突变4例(3.5%);51例患者(45.1%)有1种以上高危基因突变。MIPSS70-plus预后积分低危组、中危组、高危组、极高危组分别为28例(26.7%)、20例(19.0%)、41例(39.0%)、16例(15.3%),2年预期总生存率分别为100%、89.7%(95%CI 76.2%~100.0%)、64.8%(95%CI 47.0%~82.6%)、35.0%(95%CI 10.3%~59.7%)(P<0.001)。MIPSS70-plus的-2log似然比显著低于DIPSS(86.355对95.990,P=0.001),表明MIPSS70-plus较DIPSS有更准确的预后分组预测效能。结论 MIPSS-70plus较DIPSS预后积分系统对中国PMF患者有更好的预后评估效能。

Objective To evaluate the prognostic value of MIPSS70-plus in Chinese patients with primary myelofibrosis(PMF).Methods 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.Results 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%(n=63),CALR exon9 in 17.7%(including 15 CALR type 1 and 5 CALR type 2,n=20),MPLW515 in 4.4%(n=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%(n=51)of patients,with ASXL1 in 38.9%(n=44),SRSF2 in 7.1%(n=8),IDH1/2 in 4.4%(n=5),and EZH2 in 3.5%(n=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%CI 76.2%-100.0%)in intermediate risk,64.8%(95%CI 47.0%-82.6%)in high risk,and 35.0%(95%CI 10.3%-59.7%)in very-high risk categories,which had a significant difference(P<0.001).A significantly higher predictive power for survival of the MIPSS70-plus group was observed compared with the DIPSS group(P=0.001,-2 log-likelihood ratios of 86.355 vs 95.990 for the MIPSS70-plus and DIPSS systems,respectively).Conclusion The MIPSS70-plus had significantly higher predictive power than the DIPSS.