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儿童B细胞急性淋巴细胞白血病诊断时中枢神经系统白血病的预后意义:来自华南儿童白血病协作组的报告

Prognostic significance of CNSL at diagnosis of childhood B-cell acute lymphoblastic leukemia: A report from the South China Children's Leukemia Group.

作者信息

Xu Lu-Hong, Geng Xu, Liao Ning, Yang Li-Hua, Mai Hui-Rong, Wan Wu-Qing, Huang Li-Bin, Zheng Min-Cui, Tian Chuan, Chen Hui-Qin, Chen Qi-Wen, Long Xing-Jiang, Zhen Zi-Jun, Liu Ri-Yang, Li Qiao-Ru, Wu Bei-Yan, Wang Li-Na, Kong Xian-Ling, Chen Guo-Hua, Fang Jian-Pei, Li Yang

机构信息

Department of Pediatric Hematology/Oncology, Children's Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China.

Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China.

出版信息

Front Oncol. 2022 Aug 10;12:943761. doi: 10.3389/fonc.2022.943761. eCollection 2022.

Abstract

OBJECTIVES

The prognostic significance of acute lymphoblastic leukemia (ALL) patients with central nervous system leukemia (CNSL) at diagnosis is controversial. We aimed to determine the impact of CNSL at diagnosis on the clinical outcomes of childhood B-cell ALL in the South China Children's Leukemia Group (SCCLG).

METHODS

A total of 1,872 childhood patients were recruited for the study between October 2016 and July 2021. The diagnosis of CNSL depends on primary cytological examination of cerebrospinal fluid, clinical manifestations, and imaging manifestations. Patients with CNSL at diagnosis received two additional courses of intrathecal triple injections during induction.

RESULTS

The frequency of CNLS at the diagnosis of B-cell ALL was 3.6%. Patients with CNSL at diagnosis had a significantly higher mean presenting leukocyte count ( = 0.002) and poorer treatment response (0.05) compared with non-CNSL patients. Moreover, CNSL status was associated with worse 3-year event-free survival ( = 0.030) and a higher risk of 3-year cumulative incidence of relapse( = 0.008), while no impact was observed on 3-year overall survival ( = 0.837). Multivariate analysis revealed that CNSL status at diagnosis was an independent predictor with a higher cumulative incidence of relapse (hazard ratio = 2.809, = 0.016).

CONCLUSION

CNSL status remains an adverse prognostic factor in childhood B-cell ALL, indicating that additional augmentation of CNS-directed therapy is warranted for patients with CNSL at diagnosis.

摘要

目的

急性淋巴细胞白血病(ALL)患者诊断时合并中枢神经系统白血病(CNSL)的预后意义存在争议。我们旨在确定诊断时CNSL对华南儿童白血病协作组(SCCLG)中儿童B细胞ALL临床结局的影响。

方法

2016年10月至2021年7月期间,共招募了1872例儿童患者进行研究。CNSL的诊断取决于脑脊液的初次细胞学检查、临床表现和影像学表现。诊断时合并CNSL的患者在诱导期额外接受两个疗程的鞘内三联注射。

结果

B细胞ALL诊断时CNSL的发生率为3.6%。与非CNSL患者相比,诊断时合并CNSL的患者平均初诊白细胞计数显著更高( = 0.002),治疗反应更差( = 0.05)。此外,CNSL状态与3年无事件生存率较差( = 0.030)和3年累积复发风险较高( = 0.008)相关,而对3年总生存率无影响( = 0.837)。多变量分析显示,诊断时的CNSL状态是复发累积发生率较高的独立预测因素(风险比 = 2.809, = 0.016)。

结论

CNSL状态仍然是儿童B细胞ALL的不良预后因素,这表明对于诊断时合并CNSL的患者,有必要额外加强针对中枢神经系统的治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c36f/9399517/5f8122b91b2e/fonc-12-943761-g001.jpg

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