儿童高白细胞急性淋巴细胞白血病临床特点及预后分析  

Clinical characteristics and prognosis analysis of pediatric acute lymphoblastic leukemia with hyperleukocytosis

作　　者：陆文琪 魏彰悦 万琳 Lu Wenqi;Wei Zhangyue;Wan Lin(Department of Hematology,Children's Hospital of Soochow University,National Clinical Research Center for Hematologic Diseases,Suzhou 215124,China)

机构地区：[1]苏州大学附属儿童医院血液科、国家血液系统疾病临床医学研究中心,苏州215124

出　　处：《白血病.淋巴瘤》2025年第1期24-29,共6页Journal of Leukemia & Lymphoma

基　　金：国家自然科学基金(81800185)。

摘　　要：目的探讨儿童高白细胞急性淋巴细胞白血病(HL-ALL)临床特点及预后。方法回顾性病例系列研究。回顾性分析2016年1月至2023年4月苏州大学附属儿童医院153例初诊HL-ALL患儿临床资料。记录患儿临床特征、细胞遗传学及分子生物学特点、免疫分型、治疗反应及预后。根据免疫分型将患儿分为B系组与T系组;比较两组患儿临床特征;采用Kaplan-Meier法分析两组患儿总生存(OS),并进行log-rank检验;采用Cox比例风险模型分析OS的影响因素。结果153例患儿中,105例检出与疾病相关的融合基因,其中费城染色体阳性(Ph+)/费城染色体样(Ph-like)31例。诱导期出现肿瘤溶解综合征(TLS)28例,肺部感染53例,颅内出血6例,败血症12例。免疫分型86例为B系,67例为T系。B系组与T系组患儿性别及年龄比较,差异均有统计学意义(均P<0.05);B系组凝血功能异常发生率和血红蛋白、乳酸脱氢酶、尿酸水平均低于T系组(均P<0.01);B系组Ph+/Ph-like、MLL重排、E2A-PBX1、TEL-AML阳性率均高于T系组(均P<0.05),SIL-TAL阳性率低于T系组(P<0.05);在早期并发症方面,B系组TLS发生率低于T系组(P<0.05)。随访至2024年4月30日,中位随访时间[M(Q1,Q3)]为1150(460,2000)d,B系和T系组患儿3年OS率分别为80.6%和67.1%;两组患儿OS比较,差异无统计学意义(P=0.168)。单因素Cox分析结果显示,白细胞计数、Ph+/Ph-like阳性、发生TLS、肺部感染、颅内出血是B系HL-ALL患儿OS的影响因素(均P<0.05);白细胞计数、凝血功能异常是T系HL-ALL患儿OS的影响因素(均P<0.05)。结论HL-ALL中B系和T系组患儿性别、年龄、实验室指标及遗传学方面存在差异。白细胞计数、Ph+/Ph-like阳性、发生TLS、肺部感染、颅内出血是影响B系HL-ALL患儿OS的因素;白细胞计数、凝血功能异常是影响T系HL-ALL患儿OS的因素。Objective To explore the clinical characteristics and prognosis of pediatric acute lymphoblastic leukemia with hyperleukocytosis(HL-ALL).Methods A retrospective case series study was conducted.Clinical data of 153 children with initial diagnosis of HL-ALL from January 2016 to April 2023 in Children's Hospital of Soochow University were retrospectively analyzed.The clinical characteristics,cytogenetic and molecular biological features,immunophenotyping,treatment response and prognosis of the children were recorded.The children were divided into B-lineage and T-lineage groups according to immunophenotyping;the clinical characteristics of the two groups were compared;the overall survival(OS)of children in the two groups was analyzed by the Kaplan-Meier method,and the log-rank test was performed;the factors affecting OS were analyzed by using the Cox proportional hazards model.Results Among the 153 children,105 cases were detected with disease-related fusion genes,including 31 cases of Philadelphia chromosome-positive(Ph+)/Philadelphia chromosome-like(Ph-like).Tumor lysis syndrome(TLS)was present during induction therapy in 28 cases,pulmonary infection in 53 cases,intracranial hemorrhage in 6 cases,and sepsis in 12 cases.Immunophenotyping showed 86 cases were lineage B and 67 cases were lineage T.There were statistically significant differences in gender and age of the children between the B-lineage and T-lineage groups(both P<0.05);the incidence of coagulation abnormalities and the levels of hemoglobin,lactate dehydrogenase and uric acid in the B-lineage group were lower than those in the T-lineage group(all P<0.001).The positive rates of Ph+/Ph-like,MLL rearrangement,E2A-PBX1 and TEL-AML in the B-lineage group were higher than those in the T-lineage group(all P<0.05),while the positive rates of SIL-TAL was lower than that in the T-lineage group(P<0.05).In terms of early complications,the incidence of TLS in the B-lineage group was lower than that in the T-lineage group(P<0.05).The median follow-up time[M(Q1,Q3)]was

关 键 词：白血病 淋巴细胞 急性 高白细胞性 生存 儿童 

分 类 号：R733.71[医药卫生—肿瘤]