摘要目的 评价细胞遗传学指标对于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439例PMF患者的临床资料,应用Kaplan-Meier、Log-rank、似然比检验和COX回归模型对动态国际预后积分系统(DIPSS)、DIPSS-plus和DIPSS-Chinese进行评估.结果 439例PMF患者中男298例,女141例,中位年龄56(8～83)岁.DIPSS-plus可对中国PMF患者进行准确的危险度分组和预后评估(P＜0.01);DIPSS-plus较DIPSS有更准确的预测能力[-2 log似然比分别为989.5(DIPSS-plus)和1001.9(DIPSS),P=0.006].单因素分析显示伴有正常核型、复杂非单体核型、单纯+8和单纯平衡易位核型的患者预后较好,将染色体核型分为预后良好组(正常核型、复杂非单体核型、单纯+8和单纯平衡易位)和预后不良组(其他核型异常).经多因素分析提出了修订的DIPSS-Chinese预后积分系统,各参数赋值如下:DIPSS-Chinese低危组0分,中危组1分,高危组2.5分,不良染色体核型组0.5分,良好组0分.439例患者中低危组(0分)163例,中危-1组(0.5～1分)187例,中危-2组(1.5～2.5分)82例,高危组(3分)7例,各组中位生存期分别为74(95％ CI 42～106)、39(95％CI26～52)、12(95％CI1～25)个月,总生存差异具有统计学意义(P＜0.01).结论 在中国PMF患者中,DIPSS-plus较DIPSS有更好的预测价值,结合中国患者细胞遗传学特征修订的DIPSS-Chinese可望更好地指导PMF患者分组治疗策略的制定.

abstractsObjective To evaluate the prognostic value of cytogenetics in Chinese with primary myelofibrosis (PMF).Methods Four hundred and thirty-nine Chinese patients with PMF were retrospectively analyzed.The Kaplan-Meier method,the Log-rank test,the likelihood ratio test and the COX proportional hazards regression model were used to evaluate the prognostic scoring systems.Results Four hundred and thirty-nine Chinese patients with PMF were analyzed with a median age of 56 years (range:8-83),including 298 males and 141 females.The DIPSS-plus system could effectively evaluate prognosis in Chinese patients with PMF.There was significantly higher predictive power for survival for the DIPSS-plus group compared with the DIPSS group (P=0.006,-2 log-likelihood ratios of 989.5 and 1001.9 for the DIPSS-plus and DIPSS systems,respectively).Univariate analysis indicated that the patients with a normal karyotype,a complex karyotype that was not a monosomal karyotype,+ 8 only or a balanced translocation only had better survival.Following two cytogenetic risk categories were constructed:favorable karyotype including subiects with a normal karyotype,a complex karyotype that was not a monosomal karyotype,+ 8 only or a balanced translocation only and unfavorable karyotype included all others.The modified DIPSS-Chinese prognostic model was proposed by adopting cytogenetic categories and DIPSS-Chinese risk group.The median survival of patients classified in low risk (163 subjects),intermediate-1 risk (187 subjects),intermediate-2 risk (82 subjects) and high risk (7 subjects) were not reached,74 (95％ CI 42-106),39 (95％ CI 26-52) and 12(95％ CI 1-25)months,respectively,and there was a statistically significant difference in overall survival among the four risk groups (P＜0.01).Conclusion The DIPSS-plus had significantly higher predictive power than the DIPSS group in Chinese patients with PMF and the modified DIPSS-Chinese system based on the cytogenetic features of Chinese patients was proposed and worked well for prognostic indication.