血清sFas与sFasL在继发性噬血细胞性淋巴组织细胞增多症患者中的表达及临床意义  

Expression and Clinical Significance of Serum sFas/sFasL in Patients with Secondary Hemophagocytic Lymphohistiocytosis

作　　者：王菱菱[1,2] 程婉莹 王菊娟 尹光丽 段丽敏 田甜 仇红霞 WANG Ling-Ling;CHEN Wan-Ying;WANG Ju-Juan;YIN Guang-Li;DUAN Li-Min;TIAN Tian;QIU Hong-Xia(Department of Hematology,The First Afiliated Hospital of Nanjing Medical University,Jiangsu Province Hospital,Nanjing 210029 Jiangsu Province,China;Department of Hematology,Wuxi People's Hospital,Nanjing Medical University,Wuxi 214023,Jiangsu Province,China;Department of Geriatric Hematology,The First Affliated Hospital of Nanjing Medical University,Jiangsu Province Hospital,Nanjing 210029,Jiangsu Province,China)

机构地区：[1]南京医科大学第一附属医院江苏省人民医院血液科,江苏南京210029 [2]南京医科大学附属无锡人民医院血液科,江苏无锡214023 [3]南京医科大学第一附属医院江苏省人民医院老年血液科,江苏南京210029

出　　处：《中国实验血液学杂志》2023年第3期889-895,共7页Journal of Experimental Hematology

基　　金：国家自然科学基金(81570175)。

摘　　要：目的:探讨可溶性Fas(sFas)和sFasL在继发性噬血细胞性淋巴组织细胞增多症(sHLH)患者中的表达及临床意义。方法:选择2015年9月至2020年12月江苏省人民医院血液科收治的符合HLH-2004诊断标准的sHLH患者86例,按病因分为肿瘤相关HLH(MAHLH)组(55例)和非肿瘤相关HLH(Non-MAHLH)组(31例)。并选择30例健康体检者为正常对照组,20例系统性红斑狼疮(SLE)患者为疾病对照组。采用ELISA方法检测各组患者血清中sFas和sFasL的表达水平,同时收集患者临床资料进行统计分析,通过ROC曲线分析sFas及sFasL在sHLH中的意义。结果:初诊sHLH患者血清sFas和sFasL水平明显高于疾病对照组及正常对照组(P<0.01)。MAHLH组sFas和sFasL水平较非MAHLH(感染相关HLH和自身免疫性疾病相关HLH)组明显升高(P<0.01)。17例初治sHLH患者(17/86)治疗后的血清sFas及sFasL水平均较治疗前明显降低(P<0.01)。初诊sHLH患者血清sFas水平与SF(r=0.35)、sCD25(r=0.79)和sFasL(r=0.73)呈正相关,sFasL水平与SF(r=0.39)、sCD25(r=0.64)和sFas(r=0.73)呈正相关。与Non-MAHLH组相比,由sFas水平确诊MAHLH的最佳临界值为12743 pg/ml,敏感性及特异性分别为70.9%及71%,ROC曲线下面积为0.707(95%CI:0.593-0.821)(P<0.01)。与Non-MAHLH组相比,由sFasL水平确诊MAHLH的最佳临界值为3155 pg/ml,敏感性及特异性分别为74.5%及67.7%,ROC曲线下面积为0.765(95%CI:0.659-0.87)(P<0.01)。sFas高表达组(≥16798.5 pg/ml)和sFasL高表达组(≥4785 pg/ml)患者中位总生存时间较低表达组明显缩短(P<0.001)。结论:血清sFas和sFasL水平可用于sHLH疾病早期诊断和病因鉴别诊断,是sHLH不良预后的相关因素。Objective:To investigate the expression and clinical significance of soluble Fas(sFas) and sFasL in patients with secondary hemophagocytic lymphohistiocytosis(sHLH).Methods:From September 2015 to December2020,86 sHLH patients who met the HLH-2004 diagnostic criteria were collected.They were divided into 55 cases in the MAHLH group and 31 cases in the Non-MAHLH group according to the etiology.Thirty healthy persons were chosen as the normal control group,and 20 patients with systemic lupus erythematosus(SLE) were chosen as the disease control group.The expression levels of sFas and sFasL in the serum of patients with each group were detected by ELISA,and the clinical data were collected for statistical analysis.The significance of sFas and sFasL in sHLH was analyzed by ROC curve.Results:Serum levels of sFas and sFasL in patients with newly diagnosed sHLH were significantly higher than those in disease control group and normal control group(P<0.01).The levels of sFas and sFasL in MAHLH group were significantly higher than those in non-MAHLH(infection related HLH and autoimmune disease related HLH) group(P<0.01).The serum levels of sFas and sFasL in 17 newly treated patients with sHLH(17/86) after treatment were significantly lower than those before treatment(P<0.01).The serum sFas level in newly diagnosed sHLH patients was positively correlated with SF(r=0.35),sCD25(r=0.79) and sFasL(r=0.73).The serum sFasL level was positively correlated with SF(r=0.39),sCD25(r=0.64) and sFas(r=0.73).Compared with the Non-MAHLH group,the area under the ROC curve was 0.707(95% CI:0.593-0.821)(P=0.0015).The optimal critical value for diagnosing MAHLH by sFas level was 12 743 pg/ml,and the sensitivity and specificity were70.9% and 71% respectively.Compared with the Non-MAHLH group,the area under the ROC curve was 0.765(95%CI:0.659-0.87)(P<0.01).The median OS time of sFas high expression group(≥16798.5 pg/ml) and sFasL high expression group(≥4 785 pg/ml) was significantly shorter than that of the low expression group(P<0.001).Conc

关 键 词：SFAS SFASL 噬血细胞性淋巴组织细胞增多症 预后 

分 类 号：R551[医药卫生—血液循环系统疾病]