T细胞大颗粒淋巴细胞白血病合并纯红细胞再生障碍性贫血患者临床特征分析  

Clinical characteristic analysis of patients with T-cell large granular lymphocytic leukemia combined with pure red cell aplasia

作　　者：杜晓鹏 柴星星 庄万传 何耀 孟凡静 朱贵华 何广胜[2] 刘晓庆 Du Xiaopeng;Chai Xingxing;Zhuang Wanchuan;He Yao;Meng Fanjing;Zhu Guihua;He Guangsheng;Liu Xiaoqing(Department of Hematology,the Second People's Hospital of Lianyungang,Bengbu Medical College,Lianyungang 222000,China;Department of Hematology,Jiangsu Province Hospital,Nanjing 210029,China)

机构地区：[1]蚌埠医学院附属连云港市第二人民医院血液内科,222000 [2]江苏省人民医院血液内科,南京210029

出　　处：《白血病．淋巴瘤》2020年第8期483-487,共5页Journal of Leukemia & Lymphoma

基　　金：江苏省“六大人才高峰”高层次人才培养计划(WSN-320);江苏省“科教强卫工程”医学青年人才项目(QNRC2016495);连云港市“521高层次人才培养工程”。

摘　　要：目的提高对T细胞大颗粒淋巴细胞白血病(T-LGLL)合并纯红细胞再生障碍性贫血(PRCA)的认识。方法回顾性分析2010年8月至2019年10月就诊于蚌埠医学院附属连云港市第二人民医院和江苏省人民医院的14例初诊T-LGLL合并PRCA患者的临床特征、外周血及骨髓相关实验室检查指标。结果14例患者男性和女性各7例,中位年龄58.5岁(33~75岁);初诊时中位白细胞计数5.02×10^9/L[(1.45~8.49)×10^9/L],中位中性粒细胞绝对值1.35×10^9/L[(0.43~7.16)×10^9/L],中位淋巴细胞比例0.49(0.13~0.77),中位血红蛋白58 g/L(42~10^6 g/L),中位红细胞计数2.01×10^12/L[(0.99~3.20)×10^12/L],中位网织红细胞比例0.52(0.14~3.02),中位血小板计数96×10^9/L[(38~281)×10^9/L],中位大颗粒淋巴细胞占淋巴细胞比例71%(32%~81%);骨髓穿刺结果示:中位大颗粒淋巴细胞占有核细胞比例0.16(0.08~0.41);中位血清β2微球蛋白4.85 mg/L(2.81~7.22 mg/L);2例患者ASXL1和TET2基因突变阳性,其中1例同时有STAT3、EP300、FAM46C基因突变阳性;6例患者T细胞受体(TCR)β、γ阳性,1例TCRβ阳性,4例TCRγ阳性,1例TCRδ阳性,1例TCRβ、γ、δ均阳性,1例均阴性。8例接受环孢素治疗,6例有效;6例接受甲氨蝶呤联合激素治疗,3例有效;初始诱导有效9例,5例初始治疗无效患者行挽救治疗,2例有效。结论T-LGLL合并PRCA患者的实验室特征与单纯T-LGLL相似,以贫血为突出表现,伴有中性粒细胞或血小板减少亦常见;外周血和骨髓中易见大颗粒淋巴细胞,T淋巴细胞单克隆重排是重要特征,对免疫抑制治疗反应良好。Objective To improve the cognition of T-cell large granular lymphocytic leukemia(T-LGLL)combined with pure red cell aplasia(PRCA).Methods The clinical characteristics,peripheral blood and bone marrow laboratory indicators of 14 newly diagnosed patients with T-LGLL combined with PRCA who were admitted to the Second People's Hospital of Lianyungang Affiliated to Bengbu Medical College and the People's Hospital of Jiangsu Province from August 2010 to October 2019 were retrospectively analyzed.Results Among the 14 patients,there were 7 males and 7 females,with a median age of 58.5 years(33-75 years).At the first visit,the median white blood cell count was 5.02×10^9/L[(1.45-8.49)×10^9/L],the median absolute value of neutrophils was 1.35×10^9/L[(0.43-7.16)×10^9/L],the median lymphocyte ratio was 0.49(0.13-0.77),the median hemoglobin was 58 g/L(42-10^6 g/L),the median red blood cell count was 2.01×10^12/L[(0.99-3.20)×10^12/L],the median reticulocyte count percentage was 0.52(0.14-3.02),the median platelet was 96×10^9/L[(38-281)×10^9/L],the median large granular lymphocytes accounted for 71%(32%-81%)of lymphocytes.Bone marrow aspiration showed that the median large granular lymphocytes accounted for 0.16(0.08-0.41)of nuclear cells,and the median serumβ2 microglobulin was 4.85 mg/L(2.81-7.22 mg/L).Two patients had ASXL1 and TET2 mutations,and one of them had STAT3,EP300 and FAM46C mutations.Six patients were T cell receptor(TCR)βandγ-positive,1 patient were TCRβ-positive,4 patients were TCRγ-positive,1 patient was TCRδ-positive,1 patient was TCRβ,γandδ-positive,and 1 patient was all negative.Eight cases received cyclosporine therapy,6 cases were effective;6 cases received methotrexate combined with hormone therapy,3 cases were effective.The initial induction therapy was effective in 9 cases,5 patients who failed in the initial treatment received salvage treatment,and 2 cases were effective.Conclusions The laboratory characteristics of patients with T-LGLL combined with PRCA are similar to those of simple

关 键 词：白血病 淋巴细胞 贫血 再生障碍 纯红细胞 大颗粒淋巴细胞 

分 类 号：R733.7[医药卫生—肿瘤] R556.5[医药卫生—临床医学]