儿童急性T淋巴细胞白血病的临床特征及预后——福建地区多中心数据分析  

Clinical Features and Prognosis of Acute T-cell Lymphoblastic Leukemia in Children——Multi-Center Data Analysis in Fujian

作　　者：吴椿萍 郑湧智 李健[1] 温红[2] 翁开枝 庄树铨 吴兴国 华雪玲[1] 郑浩[1] 陈再生[1] 乐少华[1] WU Chun-Ping;ZHENG Yong-Zhi;LI Jian;WEN Hong;WENG Kai-Zhi;ZHUANG Shu-Quan;WU Xing-Guo;HUA Xue-Ling;ZHENG Hao;CHEN Zai-Sheng;LE Shao-Hua(Department of Pediatric Hematology,Fujian Medical University Union Hospital,Fujian Institute of Hematology,Fujian Provincial Key Laboratory,Fuzhou 350001,Fujian Province,China;Department of Pediatrics,The First Affiliated Hospital of Xiamen University,Xiamen 361000,Fujian Province,China;Department of Pediatric Renal Rheumatology and Immunology,Zhangzhou Affiliated Hospital of Fujian Medical University,Zhangzhou 363000,Fujian Province,China;Department of Pediatrics,Quanzhou First Hospital Affiliated 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]福建医科大学附属协和医院小儿血液科,福建省血液病研究所,福建省血液病学重点实验室,福建福州350001 [2]厦门大学附属第一医院儿科,福建厦门361000 [3]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 [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1期6-13,共8页Journal of Experimental Hematology

基　　金：国家临床重点专科建设项目(闽卫医政2021-76号);福建省恶性血液病临床医学研究中心(2020Y2006);福建医科大学启航基金项目(2019QH1032)。

摘　　要：目的:评估儿童急性T淋巴细胞白血病(T-ALL)的疗效,并探讨影响预后的危险因素。方法:回顾性分析2011年4月至2020年12月福建地区5家医院收治的127例初诊T-ALL患儿的临床资料,与同期急性前体B淋巴细胞白血病(B-ALL)患儿相比较,并采用Kaplan-Meier法分析评估患儿总生存率(OS)和无事件生存率(EFS),COX比例风险回归模型分析预后影响因素。116例规范治疗的T-ALL患儿中,78例接受CCLG-ALL 2008方案治疗,38例接受CCCG-ALL 2015方案治疗,对比两组的疗效及严重不良事件发生率。结果:T-ALL患儿中男性、年龄≥10岁、初诊白细胞数≥50×10^(9)/L、合并中枢神经系统白血病、诱导治疗中微小残留病≥1%、诱导结束时微小残留病≥0.01%的患儿比例均显著高于B-ALL患儿(P<0.05)。T-ALL患儿预期10年EFS及OS分别为59.7%和66.0%,均显著低于B-ALL患儿(P<0.001)。COX分析显示,初诊白细胞数≥100×10^(9)/L、诱导结束时未达完全缓解是更差预后的独立危险因素。CCCG-ALL 2015组与CCLG-ALL 2008组相比,感染相关严重不良事件发生率更低(15.8%vs 34.6%,P=0.042),而EFS及OS更高(73.9%vs 57.2%,PEFS=0.090;86.5%vs 62.3%,POS=0.023)。结论:T-ALL较B-ALL预后差,初诊白细胞数≥100×10^(9)/L、诱导结束时未达完全缓解(尤其是纵隔肿物未消失)为其预后不良危险因素。CCCG-ALL 2015方案可能降低感染相关严重不良事件发生率并提高疗效。Objective:To evaluate the efficacy of acute T-cell lymphoblastic leukemia(T-ALL)in children and explore the prognostic risk factors.Methods:The clinical data of 127 newly diagnosed children with T-ALL admitted to five hospitals in Fujian province from April 2011 to December 2020 were retrospectively analyzed,and compared with children with newly diagnosed acute precursor B-cell lymphoblastic leukemia(B-ALL)in the same period.Kaplan-Meier analysis was used to evaluate the overall survival(OS)and event-free survival(EFS),and COX proportional hazard regression model was used to evaluate the prognostic factors.Among 116 children with T-ALL who received standard treatment,78 cases received the Chinese Childhood Leukemia Collaborative Group(CCLG)-ALL 2008 protocol(CCLG-ALL 2008 group),and 38 cases received the China Childhood Cancer Collaborative Group(CCCG)-ALL 2015 protocol(CCCG-ALL 2015 group).The efficacy and serious adverse event(SAE)incidence of the two groups were compared.Results:Proportion of male,age≥10 years old,white blood cell count(WBC)≥50×10^(9)/L,central nervous system leukemia,minimal residual disease(MRD)≥1%during induction therapy,and MRD≥0.01%at the end of induction in T-ALL children were significantly higher than those in B-ALL children(P<0.05).The expected 10-year EFS and OS of T-ALL were 59.7%and 66.0%,respectively,which were significantly lower than those of B-ALL(P<0.001).COX analysis showed that WBC≥100×10^(9)/L at initial diagnosis and failure to achieve complete remission(CR)after induction were independent risk factors for poor prognosis.Compared with CCLG-ALL 2008 group,CCCG-ALL 2015 group had lower incidence of infection-related SAE(15.8%vs 34.6%,P=0.042),but higher EFS and OS(73.9%vs 57.2%,PEFS=0.090;86.5%vs 62.3%,POS=0.023).Conclusions:The prognosis of children with T-ALL is worse than children with B-ALL.WBC≥100×10^(9)/L at initial diagnosis and non-CR after induction(especially mediastinal mass has not disappeared)are the risk factors for poor prognosis.CCCG-ALL 2015 re

关 键 词：急性T淋巴细胞白血病 儿童 不良事件 疗效 

分 类 号：R733.71[医药卫生—肿瘤]