董玉婷, 周敏然, 李淼, 马荷花, 王冉, 秦雪梅, 陈春燕. 21例T细胞大颗粒淋巴细胞白血病的临床分析及文献回顾[J]. 中国肿瘤临床, 2020, 47(23): 1205-1209. DOI: 10.3969/j.issn.1000-8179.2020.23.186
引用本文: 董玉婷, 周敏然, 李淼, 马荷花, 王冉, 秦雪梅, 陈春燕. 21例T细胞大颗粒淋巴细胞白血病的临床分析及文献回顾[J]. 中国肿瘤临床, 2020, 47(23): 1205-1209. DOI: 10.3969/j.issn.1000-8179.2020.23.186
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Yuting Dong, Minran Zhou, Miao Li, Hehua Ma, Ran Wang, Xuemei Qin, Chunyan Chen. Clinical analysis of 21 cases of T-cell large granular lymphocytic leukemia and literature review[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2020, 47(23): 1205-1209. DOI: 10.3969/j.issn.1000-8179.2020.23.186
Citation: Yuting Dong, Minran Zhou, Miao Li, Hehua Ma, Ran Wang, Xuemei Qin, Chunyan Chen. Clinical analysis of 21 cases of T-cell large granular lymphocytic leukemia and literature review[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2020, 47(23): 1205-1209. DOI: 10.3969/j.issn.1000-8179.2020.23.186
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21例T细胞大颗粒淋巴细胞白血病的临床分析及文献回顾

Clinical analysis of 21 cases of T-cell large granular lymphocytic leukemia and literature review

    摘要
  • 摘要:
      目的  分析T细胞大颗粒淋巴细胞白血病（T-cell large granular lymphocytic leukemia，T-LGLL）患者的临床特征、治疗方案及生存情况。
      方法  回顾性分析2009年2月至2019年12月山东大学齐鲁医院收治的21例T-LGLL患者的临床及实验室检查资料，总结其临床特征、治疗方案及预后情况。
      结果  T-LGLL患者多为中老年男性，主要临床表现为乏力（61.9%）、感染（28.6%）、脾肿大（61.9%），6例患者合并自身免疫性疾病。T-LGLL患者血常规通常表现为白细胞减少（42.9%）和（或）贫血（71.4%），骨髓涂片可见大颗粒淋巴细胞增多，骨髓活检可见粒红系增生受抑，典型的T-LGLL免疫表型为CD3+ CD4- CD8+ CD57+ CD16+，88.9%患者TCR基因重排阳性。T-LGLL通常疾病进展缓慢，免疫抑制治疗总有效率为60%，血液学完全缓解率为20%，部分缓解率为40%。
      结论  T-LGLL为一种以血细胞减少、骨髓及外周血中大颗粒淋巴细胞克隆性增多为特征的疾病，常合并自身免疫性疾病，其疾病进展较慢，一线免疫抑制治疗效果尚可。

     

    Abstract:
      Objective  To summarize the clinical features, treatment options, and survival prognoses of patients with T-cell large granular lymphocytic leukemia (T-LGLL).
      Methods  The clinical and laboratory examination data of 21 patients with T-LGLL admitted to Qilu Hospital of Shandong University from February 2009 to December 2019 were retrospectively analyzed, and the clinical characteristics, treatment options, and prognosis of T-LGLL were summarized.
      Results  The majority of T-LGLL patients were middle-aged and elderly men. The main clinical manifestations included fatigue (61.9%), infection (28.6%), and splenomegaly (61.9%), and 6 cases combined with autoimmune diseases. Routine blood tests of T-LGLL patients usually revealed leukopenia (42.9%) and/or anemia (71.4%). Examination of bone marrow smears showed an increase in the number of large granular lymphocytes, and bone marrow biopsy demonstrated the suppression of granulocyte hyperplasia. The typical T-LGLL immunophenotype was CD3+ CD4- CD8+ CD57+ CD16+, with positivity for T-cell receptor gene rearrangement observed in 88.9% of cases. T-LGLL usually progresses slowly. The total effective rate of immunosuppressive therapy was 60%, with a complete hematological remission rate of 20% and a partial remission rate of 40%.
      Conclusions  T-LGLL is a disease characterized by cytopenia and a clonal increase in large granular lymphocytes in bone marrow and peripheral blood. It is often complicated by autoimmune diseases. This disease progresses slowly, and the effect of first-line immunosuppressive treatment is acceptable.

     

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