{"title":"IKZF1缺失小儿BCP-ALL的预后及强化化疗的影响：SCCLG-2016研究结果。","authors":"Shaofen Lin, Ning Liao, Xinyu Li, Lihua Yang, Yun-yan He, Yan-Lai Tang, Wu-Qing Wan, Wenguang Jia, Ya-jie Zhang, Qian Kong, Xingjiang Long, Xiang Lan, Ya-yun Ling, Danna Lin, Xiao-li Zhang, Chuan Wen, Chi-kong Li, Hong-gui Xu","doi":"10.1111/ejh.14245","DOIUrl":null,"url":null,"abstract":"<div>\n \n \n <section>\n \n <h3> Background</h3>\n \n <p>IKZF1 deletion (IKZF1<sup>del</sup>) is associated with poor prognosis in B-cell precursor acute lymphoblastic leukemia (BCP-ALL). But the prognosis of IKZF1<sup>del</sup> combined with other prognostic stratification factors remains unclear. Whether intensified treatment improves BCP-ALL prognosis has not been determined.</p>\n </section>\n \n <section>\n \n <h3> Method<b>s</b></h3>\n \n <p>A retrospective analysis was performed on 1291 pediatric patients diagnosed with BCP-ALL and treated with the South China Children's Leukemia 2016 protocol. Patients were stratified based on IKZF1 status for comparison of characteristics and outcome. Additionally, IKZF1<sup>del</sup> patients were further divided based on chemotherapy intensity for outcome assessments.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>The BCP-ALL pediatric patients with IKZF1<sup>del</sup> in south China showed poorer early response. Notably, the DFS and OS for IKZF1<sup>del</sup> patients were markedly lower than IKZF1<sup>wt</sup> group (3-year DFS: 88.7% [95% CI: 83.4%–94.0%] vs. 93.5% [95% CI: 92.0%–94.9%], <i>P</i> = .021; 3-year OS: 90.7% [95% CI: 85.8% to 95.6%] vs. 96.1% [95% CI: 95% to 97.2%, <i>P</i> = .003]), with a concurrent increase in 3-year TRM (6.4% [95% CI: 2.3%–10.5%] vs. 2.9% [95% CI: 1.9%–3.8%], <i>P</i> = .025). However, the 3-year CIR was comparable between the two groups (5.7% [95% CI: 1.8%–9.5%] vs. 3.7% [95% CI: 2.6%–4.7%], <i>P</i> = .138). Subgroup analyses reveal no factor significantly influenced the prognosis of the IKZF1<sup>del</sup> cohort. Noteworthy, intensive chemotherapy improved DFS from 85.7% ± 4.1% to 94.1% ± 0.7% in IKZF1<sup>del</sup> group (<i>P</i> = .084). Particularly in BCR::ABL positive subgroup, the 3-year DFS was remarkably improved from 53.6% ± 20.1% with non-intensive chemotherapy to 100% with intensive chemotherapy (<i>P</i> = .026).</p>\n </section>\n \n <section>\n \n <h3> Conclusions</h3>\n \n <p>Pediatric BCP-ALL patients with IKZF1<sup>del</sup> in South China manifest poor outcomes without independent prognostic significance. While no factor substantially alters the prognosis in the IKZF1<sup>del</sup> group. Intensified chemotherapy may reduce relapse rates and improve DFS in patients with IKZF1<sup>del</sup> subset, particularly in IKZF<sup>del</sup> patients with BCR::ABL positive.</p>\n </section>\n </div>","PeriodicalId":2,"journal":{"name":"ACS Applied Bio Materials","volume":null,"pages":null},"PeriodicalIF":4.6000,"publicationDate":"2024-06-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Prognosis of pediatric BCP-ALL with IKZF1 deletions and impact of intensive chemotherapy: Results of SCCLG-2016 study\",\"authors\":\"Shaofen Lin, Ning Liao, Xinyu Li, Lihua Yang, Yun-yan He, Yan-Lai Tang, Wu-Qing Wan, Wenguang Jia, Ya-jie Zhang, Qian Kong, Xingjiang Long, Xiang Lan, Ya-yun Ling, Danna Lin, Xiao-li Zhang, Chuan Wen, Chi-kong Li, Hong-gui Xu\",\"doi\":\"10.1111/ejh.14245\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div>\\n \\n \\n <section>\\n \\n <h3> Background</h3>\\n \\n <p>IKZF1 deletion (IKZF1<sup>del</sup>) is associated with poor prognosis in B-cell precursor acute lymphoblastic leukemia (BCP-ALL). But the prognosis of IKZF1<sup>del</sup> combined with other prognostic stratification factors remains unclear. Whether intensified treatment improves BCP-ALL prognosis has not been determined.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Method<b>s</b></h3>\\n \\n <p>A retrospective analysis was performed on 1291 pediatric patients diagnosed with BCP-ALL and treated with the South China Children's Leukemia 2016 protocol. Patients were stratified based on IKZF1 status for comparison of characteristics and outcome. Additionally, IKZF1<sup>del</sup> patients were further divided based on chemotherapy intensity for outcome assessments.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Results</h3>\\n \\n <p>The BCP-ALL pediatric patients with IKZF1<sup>del</sup> in south China showed poorer early response. Notably, the DFS and OS for IKZF1<sup>del</sup> patients were markedly lower than IKZF1<sup>wt</sup> group (3-year DFS: 88.7% [95% CI: 83.4%–94.0%] vs. 93.5% [95% CI: 92.0%–94.9%], <i>P</i> = .021; 3-year OS: 90.7% [95% CI: 85.8% to 95.6%] vs. 96.1% [95% CI: 95% to 97.2%, <i>P</i> = .003]), with a concurrent increase in 3-year TRM (6.4% [95% CI: 2.3%–10.5%] vs. 2.9% [95% CI: 1.9%–3.8%], <i>P</i> = .025). However, the 3-year CIR was comparable between the two groups (5.7% [95% CI: 1.8%–9.5%] vs. 3.7% [95% CI: 2.6%–4.7%], <i>P</i> = .138). Subgroup analyses reveal no factor significantly influenced the prognosis of the IKZF1<sup>del</sup> cohort. Noteworthy, intensive chemotherapy improved DFS from 85.7% ± 4.1% to 94.1% ± 0.7% in IKZF1<sup>del</sup> group (<i>P</i> = .084). Particularly in BCR::ABL positive subgroup, the 3-year DFS was remarkably improved from 53.6% ± 20.1% with non-intensive chemotherapy to 100% with intensive chemotherapy (<i>P</i> = .026).</p>\\n </section>\\n \\n <section>\\n \\n <h3> Conclusions</h3>\\n \\n <p>Pediatric BCP-ALL patients with IKZF1<sup>del</sup> in South China manifest poor outcomes without independent prognostic significance. While no factor substantially alters the prognosis in the IKZF1<sup>del</sup> group. Intensified chemotherapy may reduce relapse rates and improve DFS in patients with IKZF1<sup>del</sup> subset, particularly in IKZF<sup>del</sup> patients with BCR::ABL positive.</p>\\n </section>\\n </div>\",\"PeriodicalId\":2,\"journal\":{\"name\":\"ACS Applied Bio Materials\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":4.6000,\"publicationDate\":\"2024-06-07\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"ACS Applied Bio Materials\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://onlinelibrary.wiley.com/doi/10.1111/ejh.14245\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"MATERIALS SCIENCE, BIOMATERIALS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"ACS Applied Bio Materials","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1111/ejh.14245","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"MATERIALS SCIENCE, BIOMATERIALS","Score":null,"Total":0}
引用次数: 0
摘要
背景：IKZF1缺失（IKZF1del）与B细胞前体急性淋巴细胞白血病（BCP-ALL）的不良预后有关。但IKZF1del与其他预后分层因素相结合的预后仍不明确。强化治疗是否能改善BCP-ALL的预后尚未确定：对1291例确诊为BCP-ALL并接受华南儿童白血病2016年方案治疗的儿童患者进行了回顾性分析。根据IKZF1状态对患者进行分层，以比较特征和预后。此外，根据化疗强度对IKZF1del患者进行进一步分层，以评估结果：结果：华南地区患有IKZF1del的BCP-ALL儿童患者早期反应较差。值得注意的是，IKZF1del患者的DFS和OS明显低于IKZF1wt组（3年DFS：88.7% [95% CI：83.4%-94.0%] vs. 93.5% [95% CI：92.0%-94.9%]，P = .021；3年OS：90.7% [95% CI：92.0%-94.9%]，P = .021）：90.7% [95% CI: 85.8% to 95.6%] vs. 96.1% [95% CI: 95% to 97.2%，P = .003]），3年TRM同时增加（6.4% [95% CI: 2.3%-10.5%] vs. 2.9% [95% CI: 1.9%-3.8%], P = .025）。不过，两组患者的 3 年 CIR 值相当（5.7% [95% CI：1.8%-9.5%] vs. 3.7% [95% CI：2.6%-4.7%]，P = .138）。亚组分析显示，没有任何因素对IKZF1del队列的预后有明显影响。值得注意的是，强化化疗将IKZF1del组的DFS从85.7%±4.1%提高到94.1%±0.7%（P = .084）。特别是在BCR::ABL阳性亚组，3年DFS显著改善，从非强化化疗的53.6%±20.1%提高到强化化疗的100%（P = .026）：结论：华南地区患有IKZF1del的小儿BCP-ALL患者预后较差，但无独立预后意义。结论：在华南地区，IKZF1del组小儿BCP-ALL患者的预后较差，且无独立预后意义。加强化疗可降低IKZF1del亚组患者的复发率并改善DFS，尤其是BCR::ABL阳性的IKZFdel患者。
Prognosis of pediatric BCP-ALL with IKZF1 deletions and impact of intensive chemotherapy: Results of SCCLG-2016 study
Background
IKZF1 deletion (IKZF1del) is associated with poor prognosis in B-cell precursor acute lymphoblastic leukemia (BCP-ALL). But the prognosis of IKZF1del combined with other prognostic stratification factors remains unclear. Whether intensified treatment improves BCP-ALL prognosis has not been determined.
Methods
A retrospective analysis was performed on 1291 pediatric patients diagnosed with BCP-ALL and treated with the South China Children's Leukemia 2016 protocol. Patients were stratified based on IKZF1 status for comparison of characteristics and outcome. Additionally, IKZF1del patients were further divided based on chemotherapy intensity for outcome assessments.
Results
The BCP-ALL pediatric patients with IKZF1del in south China showed poorer early response. Notably, the DFS and OS for IKZF1del patients were markedly lower than IKZF1wt group (3-year DFS: 88.7% [95% CI: 83.4%–94.0%] vs. 93.5% [95% CI: 92.0%–94.9%], P = .021; 3-year OS: 90.7% [95% CI: 85.8% to 95.6%] vs. 96.1% [95% CI: 95% to 97.2%, P = .003]), with a concurrent increase in 3-year TRM (6.4% [95% CI: 2.3%–10.5%] vs. 2.9% [95% CI: 1.9%–3.8%], P = .025). However, the 3-year CIR was comparable between the two groups (5.7% [95% CI: 1.8%–9.5%] vs. 3.7% [95% CI: 2.6%–4.7%], P = .138). Subgroup analyses reveal no factor significantly influenced the prognosis of the IKZF1del cohort. Noteworthy, intensive chemotherapy improved DFS from 85.7% ± 4.1% to 94.1% ± 0.7% in IKZF1del group (P = .084). Particularly in BCR::ABL positive subgroup, the 3-year DFS was remarkably improved from 53.6% ± 20.1% with non-intensive chemotherapy to 100% with intensive chemotherapy (P = .026).
Conclusions
Pediatric BCP-ALL patients with IKZF1del in South China manifest poor outcomes without independent prognostic significance. While no factor substantially alters the prognosis in the IKZF1del group. Intensified chemotherapy may reduce relapse rates and improve DFS in patients with IKZF1del subset, particularly in IKZFdel patients with BCR::ABL positive.
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