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. 2018 Dec 14;39(12):977-982.
doi: 10.3760/cma.j.issn.0253-2727.2018.12.002.

[Characteristics and prognosis in adult patients with early T-cell precursor acute lymphoblastic leukemia]

[Article in Chinese]
X Y Gong  1 , Y Wang, B C Liu, H Wei, C L Zhou, D Lin, K Q Liu, S N Wei, B F Gong, G J Zhang, Y T Liu, X L Zhao, Y Li, R X Gu, S W Qiu, Y C Mi, J X Wang
Affiliations

[Characteristics and prognosis in adult patients with early T-cell precursor acute lymphoblastic leukemia]

[Article in Chinese]
X Y Gong et al. Zhonghua Xue Ye Xue Za Zhi. .

Abstract
in English, Chinese

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 13 adult ETP-ALL patients from January 2009 to March 2017 were retrospectively analyzed and compared with non-ETP ALL patients. Results: 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 (χ(2)=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%CI 1.23-1.79) 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 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.

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-ETP ALL）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-ETP ALL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-ETP ALL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（χ(2)=26.521，P<0.001）。两组患者的中位总生存（OS）时间分别为11.33（95%CI 0~28.46）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）。两组患者的中位无事件生存（EFS）时间分别为1.51（95% CI 1.23~1.79）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（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。.

Keywords: Adult; Leukemia, T lymphocyte, acute; Prognosis.

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Figures

图1
图1. 早期前体T细胞急性淋巴细胞白血病（ETP-ALL）与Non-ETP ALL患者的总生存（A）、无事件生存（B）及无复发生存曲线（C）
图2
图2. 异基因造血干细胞移植对早期前体T细胞急性淋巴细胞白血病（ETP-ALL）（A）与Non-ETP ALL（B）总生存的影响
See this image and copyright information in PMC

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