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
Department of Hematology, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China.
Department of Hematology, Guangzhou Twelfth People's Hospital, Guangzhou 510515, China.
Zhonghua Xue Ye Xue Za Zhi. 2022 Mar 14;43(3):235-240. doi: 10.3760/cma.j.issn.0253-2727.2022.03.009.
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.
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(