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

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。

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.