继发性噬血细胞综合征患者NK细胞KIR受体及功能与CTL细胞功能的研究  

Study on The Changes of KIR Receptor and Function of NK Cells in Patients with Acquired Hemophagocytic Lymphohistiocytosis

作　　者：梅仁 蔡梦洁 戴兰[1] 沈文红[1] 朱明清[1] MEI Ren;CAI Meng-jie;DAI Lan(Jiangsu Institute of Hematology,National Clinical Research Center for Hematologic Diseases,NHC Key Laboratory of Thrombosis and Hemostasis,The First Affiliated Hospital of Soochow University,Suzhou 215006)

机构地区：[1]苏州大学附属第一医院,江苏省血液研究所,国家血液系统疾病临床医学研究中心,国家卫生健康委员会血栓与止血重点实验室,215006

出　　处：《临床输血与检验》2021年第6期760-764,共5页Journal of Clinical Transfusion and Laboratory Medicine

基　　金：国家青年自然科学基金(No.81700132)资助。

摘　　要：目的研究继发性噬血细胞综合征(继发性HLH)患者NK细胞KIR受体及功能变化与CTL细胞功能变化。方法回顾性收集本院2018年2月~2021年2月的26例继发性HLH患者CD8^(+)T细胞功能和NK细胞KIR受体及功能检测的数据,并以本院17例健康者数据作为对照,将两组数据进行统计分析。结果继发性HLH患者淋巴细胞比例和NK细胞比例均明显低于对照组(33.18±1.939 vs.18.43±2.382,P<0.0001;20.38±2.090 vs.7.654±1.634,P<0.0001),CTL细胞比例高于对照组{45.5(35.275,50.375)vs.35.2(30.2,37.9),P=0.005},而两组T细胞比例无显著差异(P=0.513);与对照组(0/17)相比,NK细胞KIR受体克隆限制性表达显著升高(7/26,P=0.0310);与对照组相比,继发性HLH组NK细胞穿孔素(Perforin)表达比例降低{93(67.35,97)vs.96.6(95.1,97.8),P=0.0165},但颗粒酶B(GranzymeB)表达比例无明显变化(P=0.8717),CTL细胞穿孔素和颗粒酶B表达比例无显著差异(P=0.3207,P=0.6219)。结论继发性HLH患者发病机制与NK细胞及CTL细胞穿孔素和颗粒酶B的表达无明显关系,穿孔素的检测有可能用于快速鉴别PRF1基因突变引起的原发性HLH和继发性HLH。Objective To explore the changes of KIR receptor and function of NK cells and CTL cells in patients with acquired hemophagocytic lymphohistiocytosis(HLH).Methods The data of CD8^(+)T cell function and KIR receptor and function of NK cells and CTL cells in 26 patients with HLH from February 2018 to February 2021 were collected retrospectively,and 17 healthy subjects were used as controls.The statistical analysis was carried out between the two groups.Results The proportion of lymphocytes and NK cells in patients with HLH were significantly lower than those in the control group(33.18±1.939 vs.18.43±2.382,P<0.0001;20.38±2.090 vs.7.654±1.634,P<0.0001).The proportion of CTL cells was higher than that in the control group{45.5(35.275,50.375)vs.35.2(30.2,37.9),P=0.005},but there was no significant difference in the proportion of T cells between the two groups(P=0.513).Compared with the control group(0/17),the gram-restricted expression of KIR receptor in NK cells increased significantly(7/26,P=0.0310);compared with the control group,the expression of Perforin in HLH group was decreased{93(67.35,97)vs.96.6(95.1,97.8),P=0.0165},but the expression of GranzymeB in the two group was no statistically different(P=0.8717).The expression proportion of Perforin and GranzymeB was not significantly different in CTL cells between the two groups(P=0.3207,P=0.6219).Conclusion The pathogenesis of secondary HLH has no significant relationship with the expression of Perforin and GranzymeB in NK cells and CTL cells.The detection of Perforin can quickly exclude the deletion of PRF1 in primary HLH.

关 键 词：继发性噬血细胞综合征 KIR受体 NK功能 CTL细胞 流式细胞术 

分 类 号：R551[医药卫生—血液循环系统疾病]