22例婴儿急性淋巴细胞白血病的临床特征及预后分析  

Analysis of clinical characteristics and prognosis of 22 cases of infant acute lymphoblastic leukemia

作　　者：朱嘉莳[1] 李红[1] 邵静波[1] 张娜[1] 陈凯[1] 邹冰 王丹[1] 蒋慧[1] ZHU Jiashi;LI Hong;SHAO Jingbo;ZHANG Na;CHEN Kai;ZOU Bing;WANG Dan;JIANG Hui(Department of Hematology and Oncology,Shanghai Children′s Hospital,Children′s Hospital Affiliated to Shanghai Jiao Tong University,Shanghai 200040,China)

机构地区：[1]上海交通大学附属儿童医院血液肿瘤,上海200040

出　　处：《中国小儿血液与肿瘤杂志》2024年第2期114-118,136,共6页Journal of China Pediatric Blood and Cancer

基　　金：上海市卫生和计划生育委员会科研课题(20204Y0471)。

摘　　要：目的 分析婴儿急性淋巴细胞白血病(IALL)的临床及生物学特点,探讨其疗效及预后相关因素。方法 回顾性分析2010年1月—2020年6月于我院初诊并进行治疗的IALL患儿22例,分析其MICM分型、转归及预后影响因素。结果 IALL占我院急性淋巴细胞白血病(ALL)总收治数的3.33%。22例IALL发病时中位年龄9(3~11)月,所有患儿均为B-ALL,10例CD10表达为阴性,12例合并MLL重排(MLLr)。诱导治疗第19天,19例(86.4%)骨髓获完全缓解。12例(54.6%)治疗期间合并严重感染,5例(22.7%)因严重感染而死亡。5例(22.7%)骨髓复发,预计3年累积复发率为29.6%±11.3%,合并MLLr患儿更易出现复发(P=0.04)。22例患儿预计3年无事件生存(EFS)率为49.0%±10.8%,预计3年总生存期(OS)率为49.7%±10.9%。初诊年龄<6月及6~12月者预计3年EFS率分别为33.3%±19.2%、54.5%±12.8%(P=0.28),CD10阴性与阳性患儿的预计3年EFS率分别为40.0%±15.5%、56.3%±14.8%(P=0.29),MLLr阳性与阴性患儿的预计3年EFS率分别为33.3%±13.6%、68.6%±15.1%(P=0.12)。结论 IALL因其特殊的临床及生物学特征,治疗相关死亡率高,复发率高,总体预后差,初诊年龄<6月龄、免疫表型CD10阴性及合并MLLr的患儿预后更差。Objective To analyze the clinical and biological characteristics of infant acute lymphoblastic leukemia(IALL),and to explore its treatment efficacy and prognostic factors.Methods A retrospective analysis was conducted on 22 children who were first diagnosed with IALL and treated in our hospital from January 2010 to June 2020,morphology,immunology,cytogenetics,and molecular biology(MICM)classification,outcomes and factors affecting prognosis were analyzed.Results IALL accounts for 3.33%of the total number of acute lymphoblastic leukemia(ALL)admitted to our hospital.The median age of 22 cases of IALL was 9 months(range3-11 months)old.All children were B cell ALL,while 10 cases(45.5%)were negative for CD10 expression,and 12 cases(54.6%)were combined with MLL rearrangement(MLLr),19 cases(86.4%)achieved bone marrow complete remission on D19 of induction therapy,12(54.6%)cases with IALL had serious infections during treatment,and 5(22.7%)cases died of infection.Five cases(22.7%)had bone marrow relapse,and the 3-year cumulative relapse rate was 29.6%±11.3%.Children with MLLr were more likely to relapse(P=0.04).The 3-year event free survival(EFS)rate of 22 IALL children was 49.0%±10.8%,and the 3-year overall survival(OS)rate was 49.7%±10.9%.The 3-year EFS rates of children at first diagnosis<6 months and 6-12 months were 33.3%±19.2%and 54.5%±12.8%respectively(P=0.28).The 3-year EFS rates of CD10-negative and CD10-positive children were 40.0%±15.5%and 56.3%±14.8%respectively(P=0.29).The 3-year EFS rates of MLLr-positive and MLLr-negative children were 33.3%±13.6%and 68.6%±15.1%respectively(P=0.12).Conclusions Due to its special clinical and biological characteristics,IALL has high treatment-related mortality,high recurrence rate,and poor overall survival.The prognosis is even worse for children who are less than 6 months old at diagnosis,CD10-negative immunophenotype,and MLLr positive.

关 键 词：婴儿 急性淋巴细胞白血病 MICM分型 MLL重排 预后 

分 类 号：R733.71[医药卫生—肿瘤]