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[IKZF1基因缺失在B细胞急性淋巴细胞白血病患者中的预后意义]

[Prognostic significance of IKZF1 gene deletions in patients with B-cell acute lymphoblastic leukemia].

作者信息

Tang B Q, Cai Z H, Lin D N, Wang Z X, Liang X J, Fan Z P, Huang F, Liu Q F, Zhou H S

机构信息

Department of Hematology, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China.

Department of Hematology, Guangzhou Twelfth People's Hospital, Guangzhou 510515, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2022 Mar 14;43(3):235-240. doi: 10.3760/cma.j.issn.0253-2727.2022.03.009.

Abstract

This study aimed to investigate the prognostic significance of IKZF1 gene deletion in patients with acute B lymphoblastic leukemia (B-ALL) . The clinical data of 142 patients with B-ALL diagnosed in Nanfang Hospital between March 2016 and September 2019 were analyzed. IKZF1 deletion was found in 36.0% of the 142 patients with B-ALL, whereas exon 4-7 deletion was found in 44.0% . White blood cell counts were higher in patients with the IKZF1 deletion (52.0% and 28.3% , =0.005) ; these patients also experienced worse effects of mid-term induction therapy (40.0% and 70.7% , <0.001) and had a higher proportion of Philadelphia chromosome-positive (52.0% and 21.7% , respectively, <0.001) . Univariate analysis revealed that the 3-year overall survival rate (OS) and event-free survival rate (EFS) in the IKZF1 deletion group were significantly lower than the IKZF1 wild-type group [ (37.1±7.3) % (54.7±5.4) % , (51.8±7.9) % (73.9±4.7) % ; =0.025, 0.013, respectively]. Multivariable analysis showed that harboring IKZF1 deletion was an adverse factor of EFS and OS (=1.744, 2.036; =0.022, 0.020, respectively) . Furthermore, the IKZF1 deletion/chemotherapy group had significantly lower 3-year OS, EFS, and disease-free survival rates than other subgroups. In the IKZF1 deletion cohort, allo-hematopoietic stem cell transplantation (HSCT) significantly improved OS and EFS compared to non-allo-HSCT[ (67.9±10.4) % (31.9±11.0) % , (46.6±10.5) % (26.7±9.7) % ; =0.005, 0.026, respectively]. Pediatric-inspired chemotherapy was unable to completely reverse the negative effect of IKZF1 deletion on prognosis. Pediatric-inspired regimen therapy combined with allo-HSCT, in contrast, significantly improved the overall prognosis of IKZF1 deletion B-ALL.

摘要

本研究旨在探讨IKZF1基因缺失在急性B淋巴细胞白血病(B-ALL)患者中的预后意义。分析了2016年3月至2019年9月在南方医院确诊的142例B-ALL患者的临床资料。在142例B-ALL患者中,36.0%发现IKZF1缺失,而44.0%发现外显子4-7缺失。IKZF1缺失患者的白细胞计数更高(分别为52.0%和28.3%,P=0.005);这些患者中期诱导治疗效果也更差(分别为40.0%和70.7%,P<0.001),且费城染色体阳性比例更高(分别为52.0%和21.7%,P<0.001)。单因素分析显示,IKZF1缺失组的3年总生存率(OS)和无事件生存率(EFS)显著低于IKZF1野生型组[(37.1±7.3)%对(54.7±5.4)%,(51.8±7.9)%对(73.9±4.7)%;P分别为0.025、0.013]。多因素分析表明,携带IKZF1缺失是EFS和OS的不良因素(风险比分别为1.744、2.036;P分别为0.022、0.020)。此外,IKZF1缺失/化疗组的3年OS、EFS和无病生存率显著低于其他亚组。在IKZF1缺失队列中,与非异基因造血干细胞移植(HSCT)相比,异基因造血干细胞移植显著提高了OS和EFS[(67.9±10.4)%对(

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b4f/9072070/82e9bf1ea109/cjh-43-03-235-g001.jpg

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