儿童t系急性淋巴细胞白血病的临床特征和预后:一项单中心研究。

Xiao-Yan Chen, Jia-Yi Wang, Hua Jiang, Wei-Na Zhang
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摘要

目的:探讨t系急性淋巴细胞白血病(T-ALL)的临床特点、预后及相关影响因素。方法:回顾性分析2015年4月至2022年12月在广州市妇女儿童医疗中心接受中国儿童肿瘤组急性淋巴母细胞白血病(CCCG-ALL)方案治疗的T-ALL患儿。结果:共纳入患儿80例,中位年龄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)。SIL/TAL1是最常见的融合基因(22%,18/80),NOTCH1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%;结论:CCCG-ALL方案患儿治疗效果较好。诱导治疗第46天非中枢神经系统-1状态和MRD≥0.01%与这些患儿预后不良相关。
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[Clinical characteristics and prognosis of children with T-lineage acute lymphoblastic leukemia: a single-center study].

Objectives: 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.

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来源期刊
中国当代儿科杂志
中国当代儿科杂志 Medicine-Pediatrics, Perinatology and Child Health
CiteScore
1.50
自引率
0.00%
发文量
5006
期刊介绍: The Chinese Journal of Contemporary Pediatrics (CJCP) is a peer-reviewed open access periodical in the field of pediatrics that is sponsored by the Central South University/Xiangya Hospital of Central South University and under the auspices of the Ministry of Education of China. It is cited as a source in the scientific and technological papers of Chinese journals, the Chinese Science Citation Database (CSCD), and is one of the core Chinese periodicals in the Peking University Library. CJCP has been indexed by MEDLINE/PubMed/PMC of the American National Library, American Chemical Abstracts (CA), Holland Medical Abstracts (EM), Western Pacific Region Index Medicus (WPRIM), Scopus and EBSCO. It is a monthly periodical published on the 15th of every month, and is distributed both at home and overseas. The Chinese series publication number is CN 43-1301/R;ISSN 1008-8830. The tenet of CJCP is to “reflect the latest advances and be open to the world”. The periodical reports the most recent advances in the contemporary pediatric field. The majority of the readership is pediatric doctors and researchers.
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