IPSS及IPSS-R对骨髓增生异常综合征患者预后评估的比较分析  

A comparable analysis of IPSS and IPSS-R for evaluating prognosis of myelodysplastic syndrome

作　　者：雷烨[1] 许晓倩[1] 杨建民[1] 章卫平[1] 宋献民[1] 程辉[1] 龚胜蓝[1] 王健民[1] 

机构地区：[1]第二军医大学附属长海医院血液内科,全军血液病研究所, 上海200433

出　　处：《中华血液学杂志》2014年第12期1090-1094,共5页Chinese Journal of Hematology

基　　金：国家自然科学基金（81090413、30871100）;卫生公益性行业科研专项（201202017）

摘　　要：目的 比较国际预后积分系统（IPSS）及修订版IPSS（IPSS-R）评估原发性骨髓增生异常综合征（MDS）患者预后的分组特点及评估效能.方法 回顾性分析159例MDS患者病例资料,按IPSS及IPSS-R进行分组,比较不同预后评估系统的预后评估效能.结果 159例MDS患者中位发病年龄44（15～80）岁.检出的染色体核型异常者占38.56％（153例中59例）,以＋8最多（59例中20例）.142例患者中有34例转化为急性白血病.多因素分析显示年龄（P=0.000）、β2微球蛋白（β2-MG）水平（P=0.009）对患者有预后意义,并证实IPSS-R亚组的显著预后价值（P值均为0.000）.IPSS低危组与中危-Ⅰ组患者生存率差异无统计学意义（P＞0.05）,其余各组间生存率差异有统计学意义（P＜ 0.05）.IPSS-R低危组与高危组、非常高危组患者生存率差异有统计学意义（P＜0.05）,中危、高危、非常高危组患者生存率差异有统计学意义（P＜0.05）.IPSS-R能够对IPSS各亚组患者进行再分层,并能够将IPSS中危-Ⅰ组区分为两群预后不同的患者.结论 中国MDS患者发病年龄、IPSS亚组及异常染色体核型分布与欧美国家有所不同,IPSS-R中等危险度及以上核型比例高于欧美国家.年龄、β2-MG水平对患者有预后意义.IPSS及IPSS-R均适用于中国人,且IPSS-R对MDS患者的预后评估效果优于IPSS.应用IPSS-R可将IPSS各亚组患者再分层,对正确评估患者预后风险、提高治疗效果有重要意义.Objective To investigate the patients＇ characteristics and efficacy of prognosis evaluation by International Prognosis Scoring System （IPSS） and Revised International Prognosis Scoring System （IPSS-R） in patients with myelodysplastic syndrome （MDS）.Methods Prognostic value of IPSS and IPSS-R was evaluated on clinical data from 159 MDS patients,according to WHO classification.Results With a median age of 44 years （range：15-80 years）,MDS patients had the frequency of 38.56% with abnormal karyotype,including the most common abnormality ＋8 （20/153,12.6%）.34 of 142 patients transformed into leukemia.Age and the level of β2 micro-globulin were the prognostic factors by multivariate analysis and IPSS-R had a better prognostic significance.The differences in cumulative survival between IPSS subgroups were significant （P 〈 0.05） except that between low-and intermediate Ⅰ-risk group （P 〉 0.05）.There were statistical differences for IPSS-R low risk group vs high or very high risk group,and intermediate risk group vs high or very high risk group （P 〈 0.05）.IPSS-R enables IPSS subgroups re-stratification and split IPSS intermediate Ⅰ-risk group into two subgroups with different prognosis.Conclusion There were significant differences in age of onset,distribution of prognosis scoring system subgroups and abnormal karyotype compared with those in Europe and America.The proportion of higher risk （worse than good karyotype） in IPSS-R was higher than that in Europe and America.Age and the level of β2 micro-globulin were prognostic factors.Both IPSS and IPSS-R were applicable in Chinese MDS patients and the latter performed better.Applying IPSS-R to re-stratify IPSS subgroups helps evaluate prognosis more accurately and improve treatment outcomes.

关 键 词：骨髓增生异常综合征 预后 预后积分系统 

分 类 号：R551.3[医药卫生—血液循环系统疾病]