Objective: 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. Methods: 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. Results: 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% vs 30.8%, P=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% vs 50.9%, P=0.010) . In comparison with the non-ETP-ALL, ETP-ALL patients had older age of onset (P<0.001) , lower WBC count (P<0.001) , lower risk of CNS involvement (10.0% vs 30.2%, P=0.009) and slightly inferior overall survival (P=0.073) . T-cell lineage markers CD1a(-), CD8(-) and CD4(-) positive patients had higher CR(1) than their corresponding negative ones (P=0.002, P=0.000, P=0.001) , while CD33(-) and CD56(-) positive patients had lower ratio of achieving CR(1) than their negative ones, respectively (P=0.035, P=0.035) . Conclusion: 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.
目的： 分析我国急性T淋巴细胞白血病/淋巴瘤（T-ALL/LBL）患者的临床特征，探究急性早期前体T淋巴细胞白血病/淋巴瘤（ETP-ALL/LBL）分型的预后意义。 方法： 回顾性分析2008年1月至2014年12月间在四川大学华西医院就诊的126例T-ALL/LBL患者临床资料，基于白血病细胞的免疫表型将其分为三组：ETP-ALL/LBL（CD1a(－)，CD8(－)，CD5(－/dim)以及一种或多种干细胞和髓系相关抗原表达）、近似ETP-ALL/LBL（除CD5(+)外其他同ETP-ALL表型特征）及非ETP-ALL/LBL（non-ETP-ALL/LBL）组，对患者的实验室指标及预后相关因素进行分析。 结果： 126例T-ALL/LBL患者中男女比例为2.5∶1，中位年龄为25（14~77）岁，ETP-ALL/LBL亚型的比例高达47.6%。T-ALL患者首次化疗完全缓解（CR(1)）率显著高于T-LBL患者（64.4%对30.8%，P=0.032）；初诊外周血WBC>50×10(9)/L的患者CR(1)率显著高于WBC ≤ 50×10(9)/L的患者（78.4%对50.9%，P=0.010）。相较于non-ETP-ALL/LBL组，ETP-ALL/LBL组患者发病年龄更大（P<0.001）、外周血WBC更低（P<0.001）、病程中中枢神经系统浸润率更低（10.0%对30.2%，P=0.009）。ETP-ALL/LBL组患者CR(1)率显著低于non-ETP-ALL/LBL组（37.3%对84.6%，P<0.001）；ETP-ALL/LBL组患者较non-ETP-ALL/LBL组患者总生存期短，但差异无统计学意义（P=0.073）。T系抗原CD1a(+)组、CD8(+)组、CD4(+)组的CR(1)率均比相应的阴性组高（P值分别为0.002、0.000、0.001），而髓系抗原CD33(+)组、CD56(+)组CR(1)率均比相应的阴性组低（P值分别为0.035、0.035）。 结论： 中国青少年和成人T-ALL患者中ETP-ALL/LBL亚型比例高，ETP-ALL/LBL属于成人ALL的高危亚型，需要更精确的诊断及新的治疗策略来改善预后。.
Keywords: Flow cytometry; Immunophenotype; Leukemia, T-cell; Leukemia, early T-cell precursor; Prognosis.