儿童亚二倍体核型前体B细胞急性淋巴细胞白血病的临床特征及预后分析  

Clinical Characteristics and Prognosis of Children with Hypodip-loid B-cell Precursor Acute Lymphoblastic Leukemia

作　　者：陈成璇[1] 翁开枝 温红[3] 庄树铨 吴兴国 郑湧智 CHEN Cheng-Xuan;WENG Kai-Zhi;WEN Hong;ZHUANG Shu-Quan;WU Xing-Guo;ZHENG Yong-Zhi(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 Afiliated Hospital of Fujian Medical University,Zhangzhou 363000,Fujian Province,China;Department of Pediatrics,The First Affiliated Hospital of Xiamen University,Xiamen 361000,Fujian Province,China;Department of Pediatric,Quanzhou First Hospital Afiliated to Fujian Medical University,Quanzhou 362000,Fujian Province,China;Department of Pediatrics,Nanping First Hospital,Nanping 353000,Fujian Province,China)

机构地区：[1]福建省血液病研究所,福建省血液病学重点实验室,福建医科大学附属协和医院,福建福州350001 [2]福建医科大学附属漳州市医院儿童血液风湿肾科,福建漳州363000 [3]厦门大学附属第一医院儿科,福建厦门361000 [4]福建医科大学附属泉州第一医院儿科,福建泉州362000 [5]福建省南平市第一医院儿科,福建南平353000

出　　处：《中国实验血液学杂志》2024年第5期1356-1364,共9页Journal of Experimental Hematology

基　　金：福建省卫生健康中青年科研重大项目(2022ZQNZD012)。

摘　　要：目的:探讨儿童亚二倍体核型前体B细胞急性淋巴细胞白血病(BCP-ALL)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例初诊BCP-ALL患儿的临床资料。根据染色体核型将患儿分为伴亚二倍体核型BCP-ALL与不伴亚二倍体核型BCP-ALL组,对比两组患儿的临床特征、早期治疗反应[诱导治疗中及诱导后的微小残留病(MRD)]及远期疗效[总生存率(OS)及无事件生存率(EFS)],并进一步探讨伴亚二倍体核型BCP-ALL的预后影响因素。结果:1287例B-ALL患儿中28例(2.2%)为亚二倍体核型。伴亚二倍体核型BCP-ALL组初诊白细胞计数≥50×10^(9)/L的患者比例显著高于不伴亚二倍体核型BCP-ALL组(P=0.004),而两组在性别比例、初诊年龄分组、早期治疗反应方面差异均无统计学意义(P>0.05)。伴亚二倍体核型BCP-ALL组的5年EFS及OS率分别为75.0%(95%CI:66.8%-83.2%)、77.8%(95%CI:69.8%-85.8%)均低于不伴有亚二倍体核型BCP-ALL组的79.6%(95%CI:78.4%-80.8%)、86.4%(95%CI:85.4%-87.5%)但差异均无统计学意义(均P>0.05)。进一步按危险度分层进行亚组分析显示,伴亚二倍体核型BCPALL组5年EFS及OS率均显著低于不伴亚二倍体核型BCP-ALL的低危(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];与除外亚二倍体核型的中危(IR)组BCP-ALL患儿相近[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];均高于高危(HR)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]。单因素分析结果显示,性别、年龄、白细胞计数及诱导治疗中对OS及EFS均无显著的影响;染色体数目<40的患儿OS更低(P=0.026),但对EFS无显著影响;诱导治疗后MRD≥0.01%是更低OS及EFS的危险因素(P分别为0.002、0.001)。结论:儿童亚二倍体核型BCP-ALL预后中等,诱导治疗后MRD≥0.01%可能为其不良预后的危险因素。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 1287 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 1287 BCP-ALL patients,28 patients(2.2%)were hypodiploid BCP-ALL.The proportion of patients with white blood cell count(WBC)≥50×10^(9)/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:5

关 键 词：亚二倍体核型 前体B细胞急性淋巴细胞白血病 预后 

分 类 号：R733.71[医药卫生—肿瘤]