儿童高白细胞性急性淋巴细胞白血病的临床特征及预后分析  

Clinical features and outcomes of childhood acute lymphoblastic leukemia with hyperleukocytosis at diagnosis

作　　者：崔东艳 徐雨婷 刘爱国[1] 张艾[1] 王雅琴[1] 尹萌萌[1] 胡群[1] CUI Dongyan;XU Yuting;LIU Aiguo;ZHANG Ai;WANG Yaqin;YIN Mengmeng;HU Qun(Department of Pediatric Hematology and Oncology,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan,430030,China)

机构地区：[1]华中科技大学同济医学院附属同济医院儿童血液病专科,武汉430030 [2]复旦大学附属儿科医院血液科,上海201102

出　　处：《临床血液学杂志》2022年第9期650-655,662,共7页Journal of Clinical Hematology

基　　金：CCCG-ALL 2015多中心协作项目(No:WHTJ-2015043)。

摘　　要：目的:分析儿童高白细胞性急性淋巴细胞白血病(ALL)的临床特征并评估预后。方法:回顾性收集2015年1月—2020年6月诊断的244例初治ALL患儿的临床资料,均按照CCCG-ALL 2015骨架方案诊疗,随访至2021年12月。以初诊白细胞计数(WBC)50×10^(9)/L为界将患儿分为高白组(47例)和非高白组(197例),比较2组间临床特征、治疗反应、总生存率及无事件生存率。结果:244例ALL患儿中,男147例,女97例,男∶女为1.5∶1.0;中位初诊年龄4.9岁;低危组132例,中危组108例,高危组4例。初诊高白细胞血症的发生率为19.3%(47/244),初诊WBC与初诊外周血幼稚细胞比例呈线性正相关(P<0.001)。与非高白组比较,高白组肝大、脾大、T系及BCR-ABL1阳性ALL患儿的比例更高(P<0.05);高白组诱导治疗第19天MRD≥1%的比例更高(P=0.003),而第46天MRD在2组中分布差异无统计学意义(P=0.170)。中位随访52个月,总复发率为14.3%,5年总生存率为(90.6%±2.0%),5年无事件生存率为(80.4%±2.8%)。与非高白组比较,高白组总复发率更高、总生存率及无事件生存率更低,差异均有统计学意义(P<0.05)。多因素Cox回归分析显示,初诊高白细胞血症、KMT2A重排及第46天MRD≥0.01%是ALL患儿无事件生存的独立危险因素(P<0.05)。结论:高白细胞ALL患儿初诊特征有肝大、脾大、较高比例的T系及BCR-ABL1,初诊外周血幼稚细胞比例更高,早期治疗反应欠佳,预后不良。Objective:To evaluate clinical features and outcomes of childhood acute lymphoblastic leukemia(ALL)with different white blood cell counts(WBC)at diagnosis.Methods:A total of 244patients with ALL were retrospectively included who were newly diagnosed from January 2015to June 2020and treated by CCCG-ALL 2015protocol.The last follow-up time-point was December 2021.Children were divided into the hyperleukocytosis group(47cases)and the non-hyperleukocytosis group(197cases)by WBC at diagnosis,and clinical features,min-imal residual disease(MRD)and outcomes between the 2groups were evaluated.Results:Among 244children,there were 147males and 97females.The ratio of males to females was 1.5∶1.0.The median age of initial diag-nosis was 4.9years.There were 132cases in the low-risk group,108cases in the medium-risk group,and 4cases in the high-risk group.The incidence of newly diagnosed hyperleukocytemia(WBC≥50×10^(9)/L)was 19.3%(47/244).There was a linear positive correlation between newly diagnosed WBC and the proportion of immature cells in peripheral blood(P<0.001).Compared with the non-hyperleukocytemia group,the proportions of children with hepatomegaly,splenomegaly,T-lineage,and BCR-ABL1positive ALL in the hyperleukocytemia group were significantly higher(P<0.05).The proportion of MRD≥1%on the 19th day of induction treatment in the hyper-leukocytemia group was significantly higher(P=0.003),while there was no significant difference in the distribu-tion of MRD on the 46th day between the 2groups(P=0.170).After a median follow-up of 52months,the overall recurrence rate was 14.3%,the 5-year overall survival rate was(90.6%±2.0%),and the 5-year event-free survival rate was(80.4%±2.8%).Compared with the non-hyperleukocytemia group,patients in the hyperleuko-cytemia group had a higher overall recurrence rate,lower overall survival rate,and event-free survival rate(P<0.05).Multivariate Cox regression analysis showed that hyperleukocytosis at diagnosis,KMT2A-rearrangement,and MRD≥0.01%on the 46th day of induction were

关 键 词：儿童 急性淋巴细胞白血病 白细胞计数 高白细胞血症 预后 

分 类 号：R733.71[医药卫生—肿瘤]