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.
Zhongguo Dang Dai Er Ke Za Zhi. 2024 Dec 15;26(12):1308-1314. doi: 10.7499/j.issn.1008-8830.2408039.
To study the clinical characteristics and prognosis of T-lineage acute lymphoblastic leukemia (T-ALL) and related prognostic factors.
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.
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). was the most common fusion gene (22%, 18/80), and 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%; <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%; <0.05).
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.
研究T系急性淋巴细胞白血病（T-ALL）的临床特征、预后及相关预后因素。
对2015年4月至2022年12月在广州市妇女儿童医疗中心采用中国儿童癌症集团急性淋巴细胞白血病（CCCG-ALL）方案治疗的T-ALL患儿进行回顾性分析。
共纳入80例患儿，中位年龄为7岁3个月，男女比例为6:1。其中，纵隔肿块患儿占20%（16/80），中枢神经系统白血病患儿占4%（3/80），睾丸白血病患儿占1%（1/69）。 是最常见的融合基因（22%，18/80）， 是最常见的突变基因（69%，37/54）。中位随访时间为52个月，5年总生存率（OS）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组的5年OS率显著低于中枢神经系统-1组（66.7%±16.1% vs 90.3%±3.8%；<0.05），诱导治疗第46天微小残留病（MRD）≥0.01%的组5年OS率显著低于MRD<0.01%的组（68.6%±13.5% vs 94.8%±3.0%；<0.05）。
采用CCCG-ALL方案治疗的患儿往往有较好的治疗效果。诱导治疗第46天的非中枢神经系统-1状态和MRD≥0.01%与这些患儿的不良预后相关。
