目的： 分析总结T细胞大颗粒淋巴细胞白血病（T-cell large granular lymphocyte leukemia，T-LGLL）患者的临床及实验室特征，探讨T-LGLL的诊断及治疗。方法： 回顾性分析2019年3月至2022年12月期间，本院收治的6例连续T-LGLL患者的临床资料，分析总结其细胞形态学、骨髓细胞免疫表型检测、基因检测结果及治疗方案，并进行随访。结果： 6例T-LGLL患者诊断时中位年龄为60岁（54~70岁）。6例患者就诊时均有贫血，其中3例需要输血，3例出现脾脏肿大，1例有淋巴结肿大。6例外周血大颗粒淋巴细胞（large granular lymphocyte，LGL）形态学特征典型，但均为低绝对值计数，中位计数1.0（0.4~1.4）×10⁹/L。骨髓细胞免疫表型检测显示，所有患者LGL细胞均来源于胸腺后成熟T细胞，其中4例表达常见的CD3⁺CD8⁺CD57⁺效应T细胞特征，2例表达少见的CD3⁺CD8⁺CD57^-记忆T细胞标记。基因检测显示，6例患者T细胞受体（T cell receptor，TCR）中均能检测到不同的单克隆片段，支持T细胞的异常克隆性。二代基因测序结果显示，6例患者中4例检测到STAT3突变。6例患者均接受免疫抑制治疗，随访显示，5例患者对治疗有反应，其中5例获得了持续的血液学缓解。结论： T-LGLL单独依靠典型的细胞形态学及LGL绝对计数，不足以对T-LGLL作出早期和准确诊断，而LGL免疫表型也存在较大的差异，所以应联合形态学、免疫学、TCR克隆分析、二代基因测序分子生物学数据的多参数综合诊断。目前患者对免疫抑制治疗反应良好。

Objective This paper aims to analyze and summarize the clinical and laboratory characteristics of patients with T-cell large granular lymphocytic leukemia (T-LGLL) and explore the diagnosis and treatment of T-LGLL. Methods A retrospective analysis was conducted on the clinical data of 6 T-LGLL patients treated at our hospital from March 2019 to December 2022. The cell morphology, bone marrow cell immunophenotyping, genetic testing results, and treatment plans were analyzed and summarized, with follow-up conducted. Results The median age at diagnosis of the 6 T-LGLL patients was 60 (range 54-70) years. All 6 patients presented with anemia at the time of consultation, with 3 requi-ring blood transfusion, 3 having splenomegaly, and 1 having lymphadenopathy. Peripheral blood LGL morphology was typical in all 6 cases, but with low absolute counts. The median count was 1.0 (range 0.4-1.4) × 10⁹/L. Bone marrow cell immunophenotyping showed that all patients’ LGL cells originated from post-thymic mature T cells. 4 patients expressed the common CD3⁺CD8⁺CD57⁺ effector T-cell markers, while 2 expressed the rare CD3⁺CD8⁺CD57^- memory T-cell markers. Genetic testing revealed monoclonal fragments in the T cell receptor (TCR) of all 6 patients, supporting the clonal abnormality. The next generation gene sequencing results showed STAT3 mutations in 4 of the 6 patients. All 6 patients received immunosuppressive therapy, and follow-up revealed that 5 patients responded to the treatment and 5 out of 6 patients achieved continuous hematological remission. Conclusions The diagnosis of T-LGLL cannot be accurately and early made solely based on typical cell morphology and absolute LGL counts. Additionally, there are significant variations in LGL immunophenotypes. Therefore, an integrated multi-parameter diagnostic approach combining morphology, immunophenotyping, TCR clonal analysis, and molecular biology data from next-generation sequencing is recommended. Currently, immunosuppressive therapy shows good treatment response.