青少年急性淋巴细胞白血病临床特征及预后分析

发布时间：2018-09-02 09:57

【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。
[Abstract]:Objective to investigate the clinical features and prognosis of juvenile acute lymphoblastic leukemia treated by (CCLG)-2008 regimen. Methods from February 2008 to December 2012, the clinical manifestations and immunological typing of young patients (10 ~ 18 years old) and children (10 years old) in the blood tumor center of Beijing Children's Hospital affiliated to Capital Medical University were analyzed. Genetic characteristics and prognosis were compared and analyzed. Results A total of 723 children were treated with CCLG-2008 regimen. Among them, 102 cases (14.11%) were adolescents. Compared with children, bone marrow immunophenotype in adolescent patients was higher than that in children (14.71vs.5.80%). Cytogenetic analysis showed that there was a tendency to increase the expression of t (922) (8.82vs.4.99%) and t (1n19) (8.82vs.5.15%) in adolescent patients. The incidence rate of t _ (12) 21 (21.74vs.8.82%) with good prognosis was significantly lower. But under the same risk group, the 5-year overall survival rate (overall survival,OS) and the event-free survival rate (event-free survival,EFS) in the middle risk group and the children group were not significantly different (92.88vs.90.86%) and (77.09vs.82.90%) respectively. It is worth noting that in the high risk group, the 5-year EFS in the adolescent group was significantly lower than that in the children group (42.78 vs 54.12%). Conclusion in spite of the adverse prognostic factors, patients with adolescent leukemia can still obtain satisfactory OS. after receiving CCLG-2008 chemotherapy regimen for childhood acute lymphoblastic leukemia. However, how to improve the prognosis of high-risk adolescents is still difficult.
【作者单位】：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
【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)
【分类号】：R733.71

【相似文献】