Gong X Y, Wang Y, Liu B C, Wei H, Zhou C L, Lin D, Liu K Q, Wei S N, Gong B F, Zhang G J, Liu Y T, Zhao X L, Li Y, Gu R X, Qiu S W, Mi Y C, Wang J X
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.
Zhonghua Xue Ye Xue Za Zhi. 2018 Dec 14;39(12):977-982. doi: 10.3760/cma.j.issn.0253-2727.2018.12.002.
To analyze the clinical, laboratory characteristics and prognosis of adult early T-cell precursor acute lymphoblastic leukemia (ETP-ALL). The clinical data of 13 adult ETP-ALL patients from January 2009 to March 2017 were retrospectively analyzed and compared with non-ETP ALL patients. 13 ETP-ALL patients (17.3%) were identified in 75 adult T-ALL patients, the median age of the patients was 35 years old (15 to 49 years) and 10 patients 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 ((2)=26.521, <0.001). The median overall survival(OS) was 11.33 (95% 0-28.46) and 25.69 (95% 11.98-39.41) months, respectively. The 3-year OS was 41.7% and 40.7%, respectively (=0.699). The median event free survival (EFS) was 1.51 (95% 1.23-1.79) and 21.36 (95% 4.67-38.04) months, respectively. The 3-year EFS was 16.7% and 39.5%, respectively (=0.002). The 3-year relapse free survival (RFS) was 53.0% and 52.0%, respectively (=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. To our knowledge, this study is the first report on characteristics and prognosis of adult ETP-ALL patients in China. At total of 13 T-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.
分析成人早期T细胞前体急性淋巴细胞白血病（ETP-ALL）的临床、实验室特征及预后。回顾性分析2009年1月至2017年3月期间13例成人ETP-ALL患者的临床资料，并与非ETP-ALL患者进行比较。在75例成人T-ALL患者中，确诊13例ETP-ALL患者（17.3%），患者中位年龄为35岁（15至49岁），男性10例（76.9%）。与非ETP-ALL患者相比，ETP-ALL患者白细胞计数、乳酸脱氢酶水平、外周血原始细胞较低，胸腺肿块发生率较低，血小板计数较高。ETP-ALL患者和非ETP-ALL患者诱导化疗1个疗程后的完全缓解（CR）率分别为33.3%和90.1%（χ²=26.521，P<0.001）。中位总生存期（OS）分别为11.33（95%CI 0 - 28.46）个月和25.69（95%CI 11.98 - 39.41）个月。3年OS率分别为41.7%和40.7%（P=0.699）。中位无事件生存期（EFS）分别为1.51（95%CI 1.23 - 1.79）个月和21.36（95%CI 4.67 - 38.04）个月。3年EFS率分别为16.7%和39.5%（P=0.002）。3年无复发生存期（RFS）分别为53.0%和52.0%（P=0.797）。多因素分析显示，中枢神经系统白血病（CNSL）和异基因造血干细胞移植（allo-HSCT）是影响T-ALL患者OS的独立危险因素，ETP-ALL不影响T-ALL的预后。据我们所知，本研究是中国关于成人ETP-ALL患者特征及预后的首次报道。共有13例T-ALL患者（17.3%）被归类为ETP-ALL。与非ETP-ALL患者相比，这些患者白血病负荷较低，诱导化疗1个疗程后的CR率较低。allo-HSCT可改善ETP-ALL的预后。
