青少年急性淋巴细胞白血病临床特征及预后分析  

Clinical characteristics and outcome of adolescent patients with acute lymphoblastic leukemia

作　　者：于皎乐 吴敏媛 张瑞东 谢静 张元元 吴颖 高超 林巍 郑胡镛 

机构地区：[1]首都医科大学附属北京儿童医院血液肿瘤中心 国家儿童医学中心 儿童血液病与肿瘤分子分型北京市重点实验室儿科学国家重点学科 儿科重大疾病研究教育部重点实验室,北京100045

出　　处：《中国实用儿科杂志》2017年第9期687-692,共6页Chinese Journal of Practical Pediatrics

基　　金：北京市医院管理局青苗人才计划(QML20161202);国家自然科学基金青年基金(81400074);北京市医院管理局登峰人才培养计划团队(DFL20151101);北京市医院管理局临床医学发展专项经费资助(ZY201404);首都卫生发展科研专项-重点攻关(首发2016-1-2091)

摘　　要：目的探讨经中国儿童白血病协作组(CCLG)-2008方案治疗的青少年急性淋巴细胞白血病患者的临床特征及预后。方法对2008年2月至2012年12月就诊于首都医科大学附属北京儿童医院血液肿瘤中心的青少年组(10~18岁)与儿童组(<10岁)患者临床表现、免疫分型、遗传学特征及预后情况等进行对比分析。结果共有723例患儿接受CCLG-2008方案治疗。其中青少年102例(14.11%)。相比儿童患者,青少年患者骨髓免疫分型具有更高T-系表达比例(14.71%vs.5.80%),且细胞遗传学特征分析提示,青少年患者中存在明确的预后不良基因表现型t(9;22)(8.82%vs.4.99%)以及t(1;19)(8.82%vs.5.15%)表达比例增高的趋势,而提示预后良好的t(12;21)(21.74%vs.8.82%)发生比例则显著减低。但在相同危险分组情况下,中危组青少年组与儿童组5年整体生存率(overall survival,OS)与无事件生存率(event-free survival,EFS)差异无统计学意义,分别为(92.88%vs.90.86%)与(77.09%vs.82.90%)。值得注意的是,在高危组中,青少年组患者5年EFS明显低于儿童组(42.78%vs.54.12%)。结论尽管存在预后不良因素,青少年白血病患者在接受儿童急性淋巴细胞白血病CCLG-2008化疗方案后仍可得到较满意的OS。但如何改善高危组青少年患者的预后则仍是当前治疗难点。Objective To summarize the clinical characteristics and outcome of adolescent patients with acute lymphoblastic leukemia （ALL） treated with Chinese Children＇s Leukemia Group （CCLG）-2008 protocol over a 5-year period from 2008 to 2013. Methods Clinical features, immunophenotypes, cytogenetic abnormalities and prognosis of adolescent patients aged 10 to 18 were compared with younger patients under 10 years old. Results During 2008-- 2012, 723 patients with ALL were enrolled in our study. About 14.11% of them were 10- to 18-year old patients. Compared to younger ones, adolescent patients demonstrated a higher incidence of T-cell lineage phenotype （14.71% vs 5.8% ）, t （9 ; 22） （8.82% vs. 4.99% ）, t （1 ; 19） （8.82% vs. 5.15% ）, and significantly lower frequency of t （12 ; 21 ） （21.74% vs. 8.82% ）. In intermediate group, the 5-year overall survival rate （OS） and event-free survival rate （EFS） of adolescent patients was compatible with paediatric patients, （92.88% vs. 90.86% ） and （77.09% vs. 82.9% ） ,respectively. However, the EFS of adolescent patients with high-risk ALL was worse than paediatric patients （42.78% vs. 54.12% ）. Conclusion Despite the increased incidence of adverse prognostic factors, the survival rate of adolescent patients has been improved when treated with paediatric ALL protocol. However, the poor prognosis of adolescent patients in the high-risk group remains to be the current challenge.

关 键 词：青少年 急性淋巴细胞白血病 临床特征 预后 

分 类 号：R72[医药卫生—儿科]