论文部分内容阅读
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%n CI 76.2%～100.0%）、64.8%（95%n CI 47.0%～82.6%）、35.0%（95%n CI 10.3%～59.7%）（n P<0.001）。MIPSS70-plus的-2log似然比显著低于DIPSS（86.355对95.990，n P＝0.001），表明MIPSS70-plus较DIPSS有更准确的预后分组预测效能。n 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 n=63) , CALR exon9 in 17.7% (including 15 CALR type 1 and 5 CALR type 2, n n=20) , MPLW515 in 4.4% (n 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 n=51) of patients, with ASXL1 in 38.9% (n n=44) , SRSF2 in 7.1% (n n=8) , IDH1/2 in 4.4% (n n=5) , and EZH2 in 3.5% (n 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% n CI 76.2% -100.0% ) in intermediate risk, 64.8% (95% n CI 47.0% -82.6% ) in high risk, and 35.0% (95% n CI 10.3% -59.7% ) in very-high risk categories, which had a significant difference (n P<0.001) . A significantly higher predictive power for survival of the MIPSS70-plus group was observed compared with the DIPSS group (n P=0.001, -2 log-likelihood ratios of 86.355 n vs 95.990 for the MIPSS70-plus and DIPSS systems, respectively) .n Conclusion:The MIPSS70-plus had significantly higher predictive power than the DIPSS.