大颗粒淋巴细胞白血病临床特征分析  

Clinical analysis of large granular lymphocytic leukemia

作　　者：李素晓 范梦珂 宋云飞 陈清江 张明智[1] 张旭东[1] LI Suxiao;FAN Mengke;SONG Yunfei;CHEN Qingjiang;ZHANG Mingzhi;ZHANG Xudong(Department of Oncology,First Affiliated Hospital of Zhengzhou University,Zhengzhou,Henan 450000,China;Department of Oncology,Xinxiang Medical College,Xiniang,Henan 453003,China)

机构地区：[1]郑州大学第一附属医院肿瘤科,河南郑州450000 [2]新乡医学院肿瘤科,河南新乡453003

出　　处：《中华肿瘤防治杂志》2023年第22期1369-1374,共6页Chinese Journal of Cancer Prevention and Treatment

基　　金：国家自然基金面上项目(82070210);河南省医学科技攻关重大项目(SBGJ202001008)。

摘　　要：目的 分析大颗粒淋巴细胞白血病(LGLL)临床特征、治疗及预后情况。方法 回顾性分析2019-10-05-2022-07-22郑州大学第一附属医院的31例LGLL患者临床特征、实验室检查、治疗方案及预后情况。采用Kaplan-meier法对患者进行生存分析。结果 31例患者中位发病年龄为64岁。常见的症状为活动后乏力及胸闷(19例,61.3%)、合并感染(21例,67.7%)和发热(4例,12.9%)。19例(61.3%)患者合并自身免疫性疾病,较常见的为纯红细胞再生障碍性贫血(PRCA),其次为类风湿性关节炎(RA)。血常规表现为贫血(29例,93.5%)、中性粒细胞减少(14例,45.2%)和淋巴细胞比例增多(19例,61.3%)。流式细胞术检测结果显示,28例患者中27例T-LGLL,1例NK-LGLL(免疫表型为CD3^(-)CD94^(+)CD56^(-)CD4^(-)CD16^(-))。将27例T-LGLL患者按照表型分为CD4^(-)CD8^(+)(24例)和CD4^(-)CD8^(-)(3例),2组临床表现相似,差异无统计学意义,均P>0.05。16例患者行T细胞抗原受体(TCR)基因重排,其中15例(93.8%)例患者TCR基因重排阳性;12例为TCRαβ+,4例为TCRγδ+。26例使用免疫抑制剂作为一线治疗,总体反应率为53.8%。纳入患者中位随访时间为15个月,随访截至2022-11-30,26例患者生存,1和5年OS率分别为89.2%和84.2%。结论 LGLL首发症状各异,主要为血液系统和免疫系统异常,不同免疫分型在临床特征上无明显差异。整体预后较好,中位生存时间较长,免疫抑制治疗仍是目前较有效的治疗方式,但需要长期维持。Objective To analyze the clinical features,treatment and prognosis of patients with large granular lymphocytic leukemia(LGLL).Method The clinical characteristics,treatment regimens and prognosis of 37patients with LGLL in the First Affiliated Hospital of Zhengzhou University from October 5,2019to July 22,2022were analyzed retrospectively.Kaplan-Meier method was used for survival analysis.Result The median age was 64years old.The common initial symptoms included fatigue and chest tightness after activity(19cases,61.3%),repeated infection(21cases,67.7%)and fever(4cases,12.9%).Nineteen patients(61.3%)had comorbidities of autoimmunity diseases,and the most common type was pure red cell aplastic anemia,followed by rheumatoid arthritis;Blood routine test of LGLL patients usually manifested as anemia(29cases,93.5%),granulocytopenia(14cases,45.2%),and increased proportion of lymphocytes(19cases,61.3%).Flow cytometry showed that 27of the 28patients had T-LGLL and 1had NK-LGLL(immunophenotype was CD3^(-)CD94^(+)CD56^(-)CD4^(-)CD16^(-)).According to phenotype,27T-LGLL patients were divided into CD4^(-)CD8^(+)(24cases)and CD4^(-)CD8^(-)(3cases).The clinical manifestations of the two groups were similar,with no statistical significance,both P>0.05.T cell antigen receptor(TCR)gene rearrangement was performed in 16patients,of which15patients(93.8%)were positive for TCR gene rearrangement.Of the 16patients,12had TCRαβ+rearrangement and4had TCRγδ+rearrangement.Twenty-six patients were treated with immunosuppressants as first-line therapy,with an overall response rate of 53.8%.As of 2022-11-30,the median follow-up time of enrolled patients was 15months,and26patients survived.The 1and 5-year OS rates were 89.2%and 84.2%,respectively.Conclusions The initial symptoms of LGLL are diverse,which are mainly hematological and immunologic functional abnormalities,with no clear difference in clinical features across immunophenotypes.The prognosis of LGLL is relatively good,and the survival time is longer.Immunosuppression remains the mo

关 键 词：T大颗粒淋巴细胞白血病 大颗粒淋巴细胞 自身免疫性疾病 免疫表型 贫血 

分 类 号：R733.7[医药卫生—肿瘤]