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 Affiliated 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 Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, Jiangsu Province, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2023 Jun;31(3):889-895. doi: 10.19746/j.cnki.issn.1009-2137.2023.03.040.
To investigate the expression and clinical significance of soluble Fas (sFas) and sFasL in patients with secondary hemophagocytic lymphohistiocytosis (sHLH).
From September 2015 to December 2020, 86 sHLH patients who met the HLH2004 diagnostic criteria were collected. They were divided into 55 cases in the MAHLH group and 31 cases in the NonMAHLH 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.
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 (<0.01). The levels of sFas and sFasL in MAHLH group were significantly higher than those in nonMAHLH (infection related HLH and autoimmune disease related HLH) group (<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 (<0.01). The serum sFas level in newly diagnosed sHLH patients was positively correlated with SF(=0.35), sCD25(=0.79) and sFasL(=0.73). The serum sFasL level was positively correlated with SF(=0.39), sCD25(=0.64) and sFas(=0.73). Compared with the NonMAHLH group, the area under the ROC curve was 0.707 (95% : 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 were 70.9% and 71% respectively. Compared with the NonMAHLH group, the area under the ROC curve was 0.765(95% : 0.659-0.87)(<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 (<0.001).
Serum levels of sFas and sFasL can be used for the early diagnosis and differential diagnosis of sHLH disease, and are the factor related to the poor prognosis of sHLH.
探讨可溶性Fas（sFas）和可溶性Fas配体（sFasL）在继发性噬血细胞性淋巴组织细胞增生症（sHLH）患者中的表达及临床意义。
收集2015年9月至2020年12月符合HLH-2004诊断标准的86例sHLH患者。根据病因将其分为巨噬细胞活化相关HLH（MAHLH）组55例和非MAHLH组31例（感染相关HLH和自身免疫性疾病相关HLH）。选取30例健康人作为正常对照组，20例系统性红斑狼疮（SLE）患者作为疾病对照组。采用酶联免疫吸附测定（ELISA）法检测各组患者血清中sFas和sFasL的表达水平，并收集临床资料进行统计学分析。通过ROC曲线分析sFas和sFasL在sHLH中的意义。
新诊断的sHLH患者血清sFas和sFasL水平显著高于疾病对照组和正常对照组（P<0.01）。MAHLH组sFas和sFasL水平显著高于非MAHLH组（感染相关HLH和自身免疫性疾病相关HLH）（P<0.01）。17例新治疗的sHLH患者（17/86）治疗后血清sFas和sFasL水平显著低于治疗前（P<0.01）。新诊断的sHLH患者血清sFas水平与铁蛋白（SF，r=0.35）、可溶性白细胞介素-2受体（sCD25，r=0.79）和sFasL（r=0.73）呈正相关。血清sFasL水平与SF（r=0.39）、sCD25（r=0.64）和sFas（r=0.73）呈正相关。与非MAHLH组相比，sFas诊断MAHLH的ROC曲线下面积为0.707（95%CI：0.593-0.821）（P=0.0015）。sFas水平诊断MAHLH的最佳临界值为12 743 pg/ml，敏感性和特异性分别为70.9%和71%。与非MAHLH组相比，sFasL诊断MAHLH的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（≥4 785 pg/ml）的中位总生存期（OS）显著短于低表达组（P<0.001）。
血清sFas和sFasL水平可用于sHLH疾病的早期诊断和鉴别诊断，是sHLH预后不良的相关因素。
