穿孔素和颗粒酶B在EBV相关噬血细胞综合征中的表达及临床意义

Expression and clinical significance of perforin and granzyme B in Epstein-Barr virus-associated hemophagocytic syndrome

目的:动态观察EB病毒相关噬血细胞综合征(EBV-HLH)患儿外周血CD8^+T细胞和NK细胞穿孔素和颗粒酶B的表达变化,探索其临床意义。方法:以传染性单核细胞增多症(IM)患儿60例、排除PRF1基因缺陷的EBV-HLH患儿30例及同期体检的健康儿童30例为研究对象,采用流式细胞术分析健康对照组、IM组以及EBV-HLH患儿治疗前后外周血中CD8^+T细胞和NK细胞穿孔素和颗粒酶B的表达。结果:EBV-HLH、IM患儿CD8^+T细胞的穿孔素和颗粒酶B的表达水平均高于健康对照组(P<0.05),以IM患儿表达水平最高(P<0.05),IM患儿NK细胞穿孔素和颗粒酶B的水平高于EBV-HLH和健康对照组(P<0.05)。与治疗前比,治疗后存活的20例EBV-HLH患儿CD8^+T细胞中穿孔素和颗粒酶B的表达下降(P<0.05),而NK细胞中穿孔素和颗粒酶B水平升高(P<0.05)。结论:EBV-HLH患儿体内CD8^+T/NK细胞中穿孔素和颗粒酶B存在差异性表达,NK细胞穿孔素和颗粒酶B的高表达有利于疾病的恢复。

Objective:To dynamically observe the changes of perforin and granzyme B in CD8^+T and NK cells in peripheral blood of children with EB virus associated hemophagocytic syndrome(EBV-HLH) and to explore its clinical significance.Methods:60 cases of infectious mononucleosis(IM),30 cases of EBV-HLH without PRF1 gene defection and 30 cases of healthy children were studied.The expression of perforin and granzyme B in CD8^+T and NK cells in peripheral blood was analyzed by flow cytometry with cell surface labeling and intracellular cytokine staining,including before and after treatment in EBV-HLH patients.Results:Compared with the normal control group,the expression level of perforin and granzyme B in CD8^+T cells in the EBV-HLH and IM children increased gradually(P<0.05).The expression of perforin and granzyme B in NK cells was significantly increased in IM children(P<0.05).However,there was no significant increase in children with EBV-HLH.The level of perforin and granzyme B in CD8^+T cells decreased in EBV-HLH children after treatment,but increased significantly in NK cells( P <0. 05). Conclusion: Perforin and granzyme B were differentially expressed in CD8^+T/NK cells in children with EBV-HLH. The high expression of perforin and granzyme in NK cells was beneficial to the recovery of the disease.