[增加化疗强度对伴有IKZF1缺失的儿童急性淋巴细胞白血病预后的影响]
[Effect of increasing the intensity of chemotherapy on the prognosis of acute lymphoblastic leukemia in children with IKZF1 deletion].
作者信息
Zheng Yong-Zhi, Li Jian, LE Shao-Hua, Zheng Hao, Hua Xue-Ling, Chen Zai-Sheng, Hu Jian-Da
机构信息
Department of Pediatric Hematology, Fujian Institute of Hematology, Fujian Provincial Key Laboratory, Fujian Medical University Union Hospital, Fuzhou 350001, China.
出版信息
Zhongguo Dang Dai Er Ke Za Zhi. 2019 Jul;21(7):690-695. doi: 10.7499/j.issn.1008-8830.2019.07.014.
OBJECTIVE
To study the clinical features of acute lymphoblastic leukemia (ALL) in children with IKAROS family zinc finger 1 (IKZF1) deletion, and to observe the effect of increasing the intensity of chemotherapy on the prognosis of this disease.
METHODS
A total of 278 children diagnosed with ALL between December 2015 and February 2018 were systematically treated according to the Chinese Children's Leukemia Group-ALL 2008 protocol (CCLG-ALL 2008). The patients were divided into an IKZF1-deleted group and a control group according to the presence or absence of IKZF1. The IKZF1-deleted group was treated with the regimen for high-risk group (HR) in the CCLG-ALL 2008 protocol, while the control group received different intensities of chemotherapy according to clinical risk classification. The clinical features and event-free survival rate (EFS) were compared between the two groups.
RESULTS
A total of 24 (8.6%) cases of 278 children were found to have large deletions of exons of the IKZF1 gene. The IKZF1-deleted group had significantly higher proportions of cases with white blood cell count ≥50×10/L at initial diagnosis, BCR-ABL1 fusion gene positive, minimal residual disease ≥10% on the 15th day of induction remission treatment, minimal residual disease-high risk and clinical risk classification-high risk compared with the control group (P<0.05). The 3-year EFS rate (76%±10%) in the IKZF1-deleted group was lower than that in the control group (84%±4%), but with no significant difference between the two groups (P=0.282). The estimated 3-year EFS rate in the IKZF1-deleted-non-HR group (actually treated with the chemotherapy regimen for HR in the CCLG-ALL 2008 protocol) was 82%±12%, which was lower than that in the control-non-HR group (86%±5%), but there was no significant difference (P=0.436).
CONCLUSIONS
ALL children with IKZF1 deletion have worse early treatment response, and increasing the intensity of chemotherapy might improve the prognosis.
目的
研究伴有IKAROS家族锌指蛋白1（IKZF1）缺失的儿童急性淋巴细胞白血病（ALL）的临床特征，并观察强化化疗对该疾病预后的影响。
方法
选取2015年12月至2018年2月期间共278例诊断为ALL的儿童，按照中国儿童白血病协作组-ALL 2008方案（CCLG-ALL 2008）进行系统治疗。根据是否存在IKZF1将患者分为IKZF1缺失组和对照组。IKZF1缺失组采用CCLG-ALL 2008方案中高危组（HR）的治疗方案，而对照组根据临床风险分类接受不同强度的化疗。比较两组的临床特征和无事件生存率（EFS）。
结果
278例儿童中共有24例（8.6%）被发现存在IKZF1基因外显子的大片段缺失。与对照组相比，IKZF1缺失组初诊时白细胞计数≥50×10⁹/L、BCR-ABL1融合基因阳性、诱导缓解治疗第15天微小残留病≥10%、微小残留病高危及临床风险分类高危的病例比例显著更高（P<0.05）。IKZF1缺失组的3年EFS率（76%±10%）低于对照组（84%±4%），但两组之间无显著差异（P=0.282）。IKZF1缺失非HR组（实际采用CCLG-ALL 2008方案中HR的化疗方案治疗）的估计3年EFS率为82%±12%，低于对照组非HR组（86%±5%），但无显著差异（P=0.436）。
结论
伴有IKZF1缺失的ALL儿童早期治疗反应较差，强化化疗可能改善预后。
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