摘要
目的分析成人早期前体T细胞急性淋巴细胞白血病(ETP-ALL)的临床、实验室特征及预后情况。方法回顾性分析2009年1月至2017年3月确诊的13例ETP-ALL患者的临床和实验室资料,并与非ETP的T-ALL(Non-ETPALL)患者进行比较。结果75例T-ALL患者中,符合ETP-ALL诊断标准患者13例(17.3%),其中男10例(76.9%),女3例(23.1%),诊断时中位年龄为35(15~49)岁。与62例Non-ETPALL患者相比,ETP-ALL患者的WBC、LDH、外周血原始细胞计数较低,胸腺肿物发生率更低,PLT水平更高。ETP-ALL与Non-ETPALL患者1个疗程CR率分别为33.3%和90.1%,差异具有统计学意义(χ2=26.521,P<0.001)。两组患者的中位总生存(OS)时间分别为11.33(95%CI0~28.46)和25.69(95%CI11.98~39.41)个月,3年OS率分别为41.7%和40.7%(P=0.699)。两组患者的中位元事件生存(EFS)时间分别为1.51(95%CI 1.23~1.79)和21.36(95%C14.67~38.04)个月,3年EFS率分别为16.7%和39.5%(P=0.002),3年元复发生存(RFs)率分别为53.0%和52.0%(P=0.797)。多因素分析显示是否为ETP-ALL并不是影响T-ALL总体预后的独立因素,而是否合并中枢神经系统白血病、是否行异基因造血干细胞移植为T-ALL的独立预后因素。结论该研究为国内首次关于成人ETP-ALL特征以及预后分析的报道。在成人T-ALL患者中,ETP-ALL占17.3%,具有起病时肿瘤负荷不高,但1个疗程化疗CR率较低的临床特点。异基因造血干细胞移植有可能克服ETP-ALL的不良预后。
Objective To analyze the clinical,laboratory characteristics and prognosis of adult early T-cell precursor acute lymphoblastic leukemia (ETP-ALL).Methods The clinical data of 13adult ETP-ALL patients from January 2009to March 2017were retrospectively analyzed and compared with non-ETP ALL patients.Results 13ETP-ALL patients (17.3%)were identified in 75adult T-ALL patients,the median age of the patients was 35years old (15to 49years)and 10patients were male (76.9%).ETP-ALL patients had lower WBC count,LDH level,blasts in peripheral blood,lower incidence of thymic mass and higher PLT count compared to non-ETP ALL patients.The CR rate after one course induction chemotherapy for ETP-ALL and non-ETP ALL patients was 33.3%and 90.1%,respectively (χ2=26.521, P <0.001).The median overall survival(OS)was 11.33(95%CI 0-28.46)and 25.69(95%CI 11.98-39.41)months,respectively.The 3-year OS was 41.7%and 40.7%,respectively (P=0.699).The median event free survival (EFS)was 1.51(95%CI1.23-1.79)and 21.36(95%CI 4.67-38.04)months,respectively.The 3-year EFS was 16.7%and 39.5%,respectively (P =0.002).The 3-year relapse free survival (RFS)was 53.0%and 52.0%,respectively (P =0.797).Multivariate analysis revealed that CNSL and allo-HSCT were independent risk factors affecting OS of T-ALL and ETP-ALL didn't affect the prognosis of T-ALL. Conclusion To our knowledge,this study is the first report on characteristics and prognosis of adult ETP-ALL patients in China.At total of 13T-ALL patients (17.3%)were classified as having ETP-ALL.These patients had a lower leukemia burden and lower CR rate after one course induction compared to non-ETP ALL patients.Allo-HSCT can improve the prognosis of ETP-ALL.
作者
弓晓媛
王迎
刘兵城
魏辉
周春林
林冬
刘凯奇
魏述宁
宫本法
张广吉
刘云涛
赵邢力
李艳
顾闰夏
邱少伟
秘营昌
王建祥
Gong Xiaoyuan;Wang Ying;Liu Bingcheng;Wei Hui;Zhou Chunlin;Lin Dong;Liu Kaiqi;Wei Shuning;Gong Benfa;Zhang Guangji;Liu Yuntao;Zhao Xingli;Li Yan;Gu Runxia;Qiu Shaowei;Mi Iqngchang;Wang Jianxiang.(State Key Laboratory of Experimental Hematology,Institute of Hematology and Blood Diseases Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Tianjin 300020,China)
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2018年第12期977-982,共6页
Chinese Journal of Hematology
基金
国家科技支撑计划(2014BA109812)
国家科技重大专项课题(2017ZX09304024)
天津市血液病临床医学研究中心(15ZXLCSY00010)
协和青年科研基金(2017320022).
