[低二倍体 B 细胞前体急性淋巴细胞白血病患儿的临床特征和预后】。]

Cheng-Xuan Chen, Kai-Zhi Weng, Hong Wen, Shu-Quan Zhuang, Xing-Guo Wu, Yong-Zhi Zheng
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Further subgroup analysis by risk stratification showed that the 5-year EFS and OS rates of the hypodiploid subgroup were significantly lower than those in the low-risk (LR) group [LR group EFS: 91.4% (95%<i>CI</i> :88.4%-93.6% ), <i>P</i> < 0.001; OS: 94.7% (95%<i>CI</i> :92.1%-96.4%), <i>P</i> < 0.001] ; it was similar to that of BCP-ALL children stratified into intermediate-risk (IR) excluding hypodiploid [IR group EFS: 79.4%(95%<i>CI</i> :74.9%-83.2%), <i>P</i> =0.343; OS: 87.3%(95%<i>CI</i> :83.6%-90.2%), <i>P</i> =0.111]; while was higher than that of EFS in HR group, but the difference was not statistically significant [HR group EFS: 58.7%(95%<i>CI</i> :52.6%-64.8%), <i>P</i> =0.178. OS: 69.9%(95%<i>CI</i> :63.5%-75.4%), <i>P</i> =0.417]. Univariate analysis showed that gender, age, white blood cell count, and MRD on middle stage of induction chemotherapy had no significant impact on OS and EFS; chromosome count< 40 was a risk factor for lower OS (<i>P</i> =0.026), but has no significant effect on EFS; MRD≥0.01% after induction therapy was a risk factor for lower OS and EFS (<i>P</i> =0.002, and 0.001, respectively).</p><p><strong>Conclusion: </strong>Children with hypodiploid BCP-ALL have an intermediate prognosis, and MRD ≥0.01% after induction chemotherapy may be a risk factors for poor prognosis.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"32 5","pages":"1356-1364"},"PeriodicalIF":0.0000,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Clinical Characteristics and Prognosis of Children with Hypodiploid B-cell Precursor Acute Lymphoblastic Leukemia].\",\"authors\":\"Cheng-Xuan Chen, Kai-Zhi Weng, Hong Wen, Shu-Quan Zhuang, Xing-Guo Wu, Yong-Zhi Zheng\",\"doi\":\"10.19746/j.cnki.issn.1009-2137.2024.05.008\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objective: </strong>To analyze the clinical characteristics and prognosis of children with hypodiploid B-cell precursor acute lymphoblastic leukemia (BCP-ALL).</p><p><strong>Methods: </strong>The clinical data of 1 287 children with BCP-ALL admitted to five hospital in Fujian province from April 2011 to December 2020 were retrospectively analyzed. 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引用次数: 0

摘要

目的分析低二倍体B细胞前体急性淋巴细胞白血病(BCP-ALL)患儿的临床特征和预后:方法:回顾性分析福建省五家医院2011年4月至2020年12月收治的1 287例BCP-ALL患儿的临床资料。根据染色体核型结果,将所有患者分为低二倍体亚组和非低二倍体亚组。比较两组患者的临床特征、早期治疗反应[诱导化疗中期和诱导化疗结束时的最小残留病(MRD)]和远期疗效[总生存期(OS)和无事件生存期(EFS)]。进一步探讨了低二倍体BCP-ALL的预后因素:在1287例BCP-ALL患者中,28例(2.2%)为低二倍体BCP-ALL。低倍体亚组患者白细胞计数(WBC)≥50×109/L的比例明显高于非低倍体亚组(P=0.004),而两组患者的性别比例、初诊年龄组别和早期治疗反应差异无统计学意义(P均>0.05)。低二倍体亚组的5年EFS和OS分别为75.0%(95%CI :66.8%-83.2%)和77.8%(95%CI :69.8%-85.8%),低于非低二倍体亚组 [EFS:79.6%(95%CI :78.4%-80.8%);OS:86.4%(95%CI :78.4%-80.8%):86.4%(95%CI :85.4%-87.5%)],但差异无统计学意义(P均大于0.05)。按风险分层进行的进一步亚组分析显示,低二倍体亚组的5年EFS和OS率明显低于低风险(LR)组 [LR组 EFS:91.4%(95%CI :88.4%-93.6%),P<0.001;OS:94.7%(95%CI :85.4%-87.5%)],但差异无统计学意义(P均>0.05):94.7%(95%CI :92.1%-96.4%),P <0.001];与分层为中危(IR)排除低二倍体的BCP-ALL儿童相似 [IR组EFS:79.4%(95%CI :74.9%-83.2%),P =0.343;OS:87.3%(95%CI :92.1%-96.4%),P <0.001]:87.3%(95%CI :83.6%-90.2%),P =0.111];而HR组的EFS高于IR组,但差异无统计学意义 [HR组EFS:58.7%(95%CI :52.6%-64.8%),P =0.178。OS:69.9%(95%CI :63.5%-75.4%),P =0.417]。单变量分析显示,性别、年龄、白细胞计数和诱导化疗中期的MRD对OS和EFS无显著影响;染色体数目< 40是降低OS的危险因素(P =0.026),但对EFS无显著影响;诱导治疗后MRD≥0.01%是降低OS和EFS的危险因素(分别为P =0.002和0.001):结论:低二倍体BCP-ALL患儿的预后处于中等水平,诱导化疗后MRD≥0.01%可能是预后不良的危险因素。
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[Clinical Characteristics and Prognosis of Children with Hypodiploid B-cell Precursor Acute Lymphoblastic Leukemia].

Objective: To analyze the clinical characteristics and prognosis of children with hypodiploid B-cell precursor acute lymphoblastic leukemia (BCP-ALL).

Methods: The clinical data of 1 287 children with BCP-ALL admitted to five hospital in Fujian province from April 2011 to December 2020 were retrospectively analyzed. According to the results of chromosome karyotype, all the patients were grouped into hypodiploid subgroup and non-hypodiploid subgroup. The clinical characteristics, early treatment response [minimal residual disease (MRD) on middle stage of induction chemotherapy and end of induction chemotherapy] and long-term efficacy [overall survival (OS) and event-free survival (EFS)] were compared. The prognostic factors of hypodiploid BCP-ALL were further explored.

Results: Among 1 287 BCP-ALL patients, 28 patients (2.2%) were hypodiploid BCP-ALL. The proportion of patients with white blood cell count (WBC)≥50×109/L in the hypodiploid subgroup was significantly higher than that in the non-hypodiploid subgroup (P =0.004), while there was no statistically significant difference in gender ratio, age group at initial diagnosis, and early treatment response between the two groups (all P >0.05). The 5-year EFS and OS rate of the hypodiploid subgroup were 75.0%(95%CI :66.8%-83.2%) and 77.8%(95%CI :69.8%-85.8%), respectively, which were lower than those of non-hypodiploid subgroup [EFS: 79.6%(95%CI :78.4%-80.8%); OS: 86.4%(95%CI :85.4%-87.5%)], but the difference was not statistically significant (all P >0.05). Further subgroup analysis by risk stratification showed that the 5-year EFS and OS rates of the hypodiploid subgroup were significantly lower than those in the low-risk (LR) group [LR group EFS: 91.4% (95%CI :88.4%-93.6% ), P < 0.001; OS: 94.7% (95%CI :92.1%-96.4%), P < 0.001] ; it was similar to that of BCP-ALL children stratified into intermediate-risk (IR) excluding hypodiploid [IR group EFS: 79.4%(95%CI :74.9%-83.2%), P =0.343; OS: 87.3%(95%CI :83.6%-90.2%), P =0.111]; while was higher than that of EFS in HR group, but the difference was not statistically significant [HR group EFS: 58.7%(95%CI :52.6%-64.8%), P =0.178. OS: 69.9%(95%CI :63.5%-75.4%), P =0.417]. Univariate analysis showed that gender, age, white blood cell count, and MRD on middle stage of induction chemotherapy had no significant impact on OS and EFS; chromosome count< 40 was a risk factor for lower OS (P =0.026), but has no significant effect on EFS; MRD≥0.01% after induction therapy was a risk factor for lower OS and EFS (P =0.002, and 0.001, respectively).

Conclusion: Children with hypodiploid BCP-ALL have an intermediate prognosis, and MRD ≥0.01% after induction chemotherapy may be a risk factors for poor prognosis.

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来源期刊
中国实验血液学杂志
中国实验血液学杂志 Medicine-Medicine (all)
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