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[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与预后]

[Clinical Characteristics and Prognosis of Children with Hypodiploid B-cell Precursor Acute Lymphoblastic Leukemia].

作者信息

Chen Cheng-Xuan, Weng Kai-Zhi, Wen Hong, Zhuang Shu-Quan, Wu Xing-Guo, Zheng Yong-Zhi

机构信息

Fujian Institute of Hematology, Fujian Provincial Key Laboratory, Fujian Medical University Union Hospital, Fuzhou 350001, Fujian Province, China.

Department of Pediatric Hematology, Rheumatology and Nephrology, Zhangzhou Affiliated Hospital of Fujian Medical University, Zhangzhou 363000, Fujian Province, China.

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Oct;32(5):1356-1364. doi: 10.19746/j.cnki.issn.1009-2137.2024.05.008.

Abstract

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)≥50×10/L in the hypodiploid subgroup was significantly higher than that in the non-hypodiploid subgroup ( =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 >0.05). The 5-year EFS and OS rate of the hypodiploid subgroup were 75.0%(95% :66.8%-83.2%) and 77.8%(95% :69.8%-85.8%), respectively, which were lower than those of non-hypodiploid subgroup [EFS: 79.6%(95% :78.4%-80.8%); OS: 86.4%(95% :85.4%-87.5%)], but the difference was not statistically significant (all >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% :88.4%-93.6% ), < 0.001; OS: 94.7% (95% :92.1%-96.4%), < 0.001] ; it was similar to that of BCP-ALL children stratified into intermediate-risk (IR) excluding hypodiploid [IR group EFS: 79.4%(95% :74.9%-83.2%), =0.343; OS: 87.3%(95% :83.6%-90.2%), =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% :52.6%-64.8%), =0.178. OS: 69.9%(95% :63.5%-75.4%), =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 ( =0.026), but has no significant effect on EFS; MRD≥0.01% after induction therapy was a risk factor for lower OS and EFS ( =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.

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)患儿的临床特征及预后。

方法

回顾性分析2011年4月至2020年12月福建省5家医院收治的1287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。比较两组的临床特征、早期治疗反应[诱导化疗中期及诱导化疗结束时的微小残留病(MRD)]及长期疗效[总生存(OS)和无事件生存(EFS)]。进一步探讨低二倍体BCP-ALL的预后因素。

结果

1287例BCP-ALL患者中,28例(2.2%)为低二倍体BCP-ALL。低二倍体亚组中白细胞计数(WBC)≥50×10⁹/L的患者比例显著高于非低二倍体亚组(P =0.004),而两组间性别比例、初诊年龄组及早期治疗反应比较,差异均无统计学意义(均P>0.05)。低二倍体亚组的5年EFS率和OS率分别为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:85.4%-87.5%)],但差异无统计学意义(均P>0.05)。按危险分层进一步亚组分析显示,低二倍体亚组的5年EFS率和OS率显著低于低危(LR)组[LR组EFS:91.4%(95%CI:88.4%-93.6%),P<0.001;OS:94.7%(95%CI:92.1%-96.4%),P<0.001];与排除低二倍体的中危(IR)BCP-ALL患儿相似[IR组EFS:79.4%(95%CI:74.9%-83.2%),P =0.343;OS:87.3%(95%CI:83.6%-90.2%),P =0.111];高于高危(HR)组的EFS,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]。单因素分析显示,性别、年龄、白细胞计数及诱导化疗中期的MRD对OS和EFS均无显著影响;染色体数<40是OS降低的危险因素(P =0.026),但对EFS无显著影响;诱导治疗后MRD≥0.01%是OS和EFS降低的危险因素(分别为P =0.002和0.001)。

结论

低二倍体BCP-ALL患儿预后中等,诱导化疗后MRD≥0.01%可能是预后不良的危险因素。

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