单中心儿童急性T淋巴细胞白血病的临床特征及预后分析  

Clinical characteristics and prognosis of children with T-lineage acute lymphoblastic leukemia:a single-center study

作　　者：陈晓燕 王嘉怡 江华 张伟娜 CHEN Xiao-Yan;WANG Jia-Yi;JIANG Hua;ZHANG Wei-Na(Department of Hematology,Guangzhou Women and Children's Medical Center,Guangzhou Medical University,Guangzhou 510000,China)

机构地区：[1]广州医科大学附属妇女儿童医疗中心血液肿瘤科,广东广州510000

出　　处：《中国当代儿科杂志》2024年第12期1308-1314,共7页Chinese Journal of Contemporary Pediatrics

基　　金：广州市妇女儿童医疗中心临床博士启动科研基金项目(2021BS008);广州市科技局一般项目(博士青年科技人员类)(2023A04J0606);广州市科技局(登峰医院)基础研究项目(2023A03J0893)。

摘　　要：目的分析急性T淋巴细胞白血病(T-acute lymphoblastic leukemia,T-ALL)的临床特征、疗效及预后因素。方法对2015年4月—2022年12月广州医科大学附属妇女儿童医疗中心使用中国儿童肿瘤协作组急性淋巴细胞白血病方案治疗的T-ALL患儿进行回顾性分析。结果共纳入80例患儿,中位年龄为7岁3个月,男∶女为6∶1,纵隔占位占20%(16/80),伴中枢神经系统白血病占4%(3/80),睾丸白血病1%(1/69)。常见融合基因为SIL/TAL1(22%,18/80),常见基因突变为NOCTH1(69%,37/54)。中位随访时间为52个月,5年总生存(overall survival,OS)率为87.3%±4.0%,5年无事件生存率为84.0%±4.3%。非中枢神经系统-1状态患儿5年OS率低于中枢神经系统-1组(66.7%±16.1%vs 90.3%±3.8%,P<0.05),第46天微小残留病≥0.01%组5年OS率低于<0.01%组(68.6%±13.5%vs 94.8%±3.0%,P<0.05)。结论采用中国儿童肿瘤协作组急性淋巴细胞白血病方案治疗儿童T-ALL疗效较好。非中枢神经系统-1状态、诱导治疗第46天微小残留病≥0.01%与患儿不良预后有关。Objective To study the clinical characteristics and prognosis of T-lineage acute lymphoblastic leukemia(T-ALL)and related prognostic factors.Methods A retrospective analysis was conducted on the children with T-ALL who were treated with the Chinese Children's Cancer Group Acute Lymphoblastic Leukemia(CCCG-ALL)regimen in Guangzhou Women and Children's Medical Center between April 2015 and December 2022.Results A total of 80 children were included,with a median age of 7 years and 3 months and a male/female ratio of 6:1.Among these children,the children with mediastinal mass accounted for 20%(16/80),those with central nervous system leukemia accounted for 4%(3/80),and those with testicular leukemia accounted for 1%(1/69).SIL/TAL1 was the most common fusion gene(22%,18/80),and NOTCH1 was the most common mutation gene(69%,37/54).The median follow-up time was 52 months,with a 5-year overall survival(OS)rate of 87.3%±4.0%and a 5-year event-free survival rate of 84.0%±4.3%.The non-central nervous system-1 group had a significantly lower 5-year OS rate than the central nervous system-1 group(66.7%±16.1%vs 90.3%±3.8%;P<0.05),and the group with minimal residual disease(MRD)≥0.01%on day 46 of induction therapy had a significantly lower 5-year OS rate than the group with MRD<0.01%(68.6%±13.5%vs 94.8%±3.0%;P<0.05).Conclusions Children treated with the CCCG-ALL regimen tend to have a good treatment outcome.Non-central nervous system-1 status and MRD≥0.01%on day 46 of induction therapy are associated with the poor prognosis in these children.

关 键 词：白血病 T淋巴细胞 预后 儿童 

分 类 号：R733.71[医药卫生—肿瘤]