Liao H Y, Sun Z Y, Wang Y X, Jin Y M, Zhu H L, Jiang N G
Department of Laboratory Medicine, West China Hospital, Sichuan University, Chengdu 610041, China.
National Center for Clinical Laboratories, Beijing 100730, China.
Zhonghua Xue Ye Xue Za Zhi. 2019 Jul 14;40(7):561-567. doi: 10.3760/cma.j.issn.0253-2727.2019.07.005.
To evaluate the clinical characteristics of T-cell acute leukemia/lymphoma (T-ALL) and explore the prognosis significance of early T-cell precursor leukemia/lymphoma. A cohort of 126 patients diagnosed with T-ALL from 2008 to 2014 in West China Hospital, Sichuan University were enrolled in this study. They were further categorized by immunophenotype according to the expression of T-cell lineage markers CD1a, CD8, CD5 and one or more stem cell or myeloid markers. The laboratory indicators and prognosis factors were also statistically analyzed. Of all patients, the ratio of male to female was 2.5∶1, with the median age of 25 years old (range 14 to 77) . The percentage of ETP-ALL was up to 47.6%. T-ALL patients showed higher ratio in first clinical remission rate (CR(1)) than T-LBL ones (64.4% 30.8%, =0.032) . Group with WBC count higher than 50×10(9)/L at presentation showed higher ration of achieving CR(1) than those lower than 50×10(9)/L (78.4% 50.9%, =0.010) . In comparison with the non-ETP-ALL, ETP-ALL patients had older age of onset (<0.001) , lower WBC count (<0.001) , lower risk of CNS involvement (10.0% 30.2%, =0.009) and slightly inferior overall survival (=0.073) . T-cell lineage markers CD1a(-), CD8(-) and CD4(-) positive patients had higher CR(1) than their corresponding negative ones (=0.002, =0.000, =0.001) , while CD33(-) and CD56(-) positive patients had lower ratio of achieving CR(1) than their negative ones, respectively (=0.035, =0.035) . Flow cytometry and associated markers for immunophenotyping was of significance in the diagnosis and prognosis monitoring of T-ALL/LBL. The percentage of ETP-ALL/LBL subtype was high in Chinese adolescent and adult T-ALL patients. ETP-ALL/LBL was a high risk subtype, which needs more precise standard for diagnosis and advanced therapies for better outcome.
评估T细胞急性白血病/淋巴瘤（T-ALL）的临床特征，并探讨早期T细胞前体白血病/淋巴瘤的预后意义。本研究纳入了2008年至2014年在四川大学华西医院确诊为T-ALL的126例患者。根据T细胞谱系标志物CD1a、CD8、CD5以及一种或多种干细胞或髓系标志物的表达，通过免疫表型对他们进行进一步分类。同时对实验室指标和预后因素进行统计学分析。所有患者中，男女比例为2.5∶1，中位年龄为25岁（范围14至77岁）。早期T细胞前体ALL（ETP-ALL）的比例高达47.6%。T-ALL患者首次临床缓解率（CR(1)）高于T淋巴母细胞淋巴瘤（T-LBL）患者（64.4%对30.8%，P = 0.032）。初诊时白细胞计数高于50×10⁹/L的组达到CR(1)的比例高于低于50×10⁹/L的组（78.4%对50.9%，P = 0.010）。与非ETP-ALL相比，ETP-ALL患者发病年龄更大（P<0.001），白细胞计数更低（P<0.001），中枢神经系统受累风险更低（10.0%对30.2%，P = 0.009），总体生存率略低（P = 0.073）。T细胞谱系标志物CD1a(-)、CD8(-)和CD4(-)阳性的患者CR(1)高于相应阴性患者（P = 0.002、P = 0.000、P = 0.001），而CD33(-)和CD56(-)阳性的患者达到CR(1)的比例分别低于其阴性患者（P = 0.035、P = 0.035）。流式细胞术及相关免疫表型标志物对T-ALL/LBL的诊断和预后监测具有重要意义。中国青少年和成人T-ALL患者中ETP-ALL/LBL亚型的比例较高。ETP-ALL/LBL是一种高危亚型，需要更精确的诊断标准和先进的治疗方法以获得更好的预后。
