摘要目的 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(CR1)率显著高于T-LBL患者(64.4％对30.8％,P=0.032);初诊外周血WBC>50×109/L的患者CR1率显著高于WBC≤50×109/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CR1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CR1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CR1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.

abstractsObjective 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 theexpression 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(CR1)than T-LBL ones(64.4％ vs 30.8％, P=0.032). Group with WBC count higher than 50×109/L at presentation showed higher ration of achieving CR1 than those lower than 50×109/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 CR1 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 CR1 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.