目的:成人发作的斯蒂尔病(AOSD)和继发性噬血细胞性淋巴组织细胞增生症(sHLH)都是高铁蛋白细胞因子风暴综合征,在住院患者中难以区分。本研究的目的是比较炎症标志物铁蛋白,D-二聚体,C反应蛋白(CRP),AOSD和sHLH患者的可溶性CD25(sCD25)。选择这四种标志物是因为它们广泛可用并且代表炎性疾病的不同方面:巨噬细胞活化(铁蛋白);内皮病(D-二聚体);白细胞介素-1/白细胞介素-6/肿瘤坏死因子升高(CRP)和T细胞活化(sCD25)。
方法:这是一项单中心回顾性研究。包括2009年至2023年由温哥华总医院血液科诊断为AOSD或sHLH的患者。
结果:确定了16例AOSD和44例sHLH患者。AOSD中的铁蛋白低于HLH(中位数11360μg/L与29020μg/L,p=0.01),而D-二聚体没有显着差异(中位数5310mg/LFEU与7000毫克/升FEU,p=.3)。CRP较高(中位数168mg/Lvs.71mg/L,p<0.01)和sCD25较低(中位数2220vs.7280U/mL,与HLH相比,AOSD中的p=.004)。使用CRP>130mg/L和sCD25<3900U/mL来区分AOSD与HLH的组合ROC曲线具有0.94(95%置信区间0.93-0.97)的曲线下面积(AUC),灵敏度91%和特异性93%。
结论:这些研究结果表明,广泛使用的实验室检查,如CRP和sCD25,可以帮助临床医生在患有严重高铁蛋白血症的急性成人患者中区分AOSD和HLH.有必要进行更大的研究,以检查更多样化的细胞因子风暴综合征中更广泛的临床上可用的炎症生物标志物。
OBJECTIVE: Adult-onset Still\'s disease (AOSD) and secondary hemophagocytic lymphohistiocytosis (sHLH) are both hyperferritinemic cytokine storm syndromes that can be difficult to distinguish from each other in hospitalized patients. The objective of this study was to compare the inflammatory markers ferritin, D-dimer, C-reactive protein (CRP), and soluble CD25 (sCD25) in patients with AOSD and sHLH. These four markers were chosen as they are widely available and represent different aspects of inflammatory diseases: macrophage activation (ferritin); endothelialopathy (D-dimer); interleukin-1/interleukin-6/tumour necrosis factor elevation (CRP) and T cell activation (sCD25).
METHODS: This was a single-center retrospective study. Patients diagnosed by the Hematology service at Vancouver General Hospital for AOSD or sHLH from 2009 to 2023 were included.
RESULTS: There were 16 AOSD and 44 sHLH patients identified. Ferritin was lower in AOSD than HLH (median 11 360 μg/L vs. 29 020 μg/L, p = .01) while D-dimer was not significantly different (median 5310 mg/L FEU vs. 7000 mg/L FEU, p = .3). CRP was higher (median 168 mg/L vs. 71 mg/L, p <.01) and sCD25 was lower (median 2220 vs. 7280 U/mL, p = .004) in AOSD compared to HLH. The combined ROC curve using CRP >130 mg/L and sCD25< 3900 U/mL to distinguish AOSD from HLH had an area under the curve (AUC) of 0.94 (95% confidence interval 0.93-0.97) with sensitivity 91% and specificity 93%.
CONCLUSIONS: These findings suggest that simple, widely available laboratory tests such as CRP and sCD25 can help clinicians distinguish AOSD from HLH in acutely ill adults with extreme hyperferritinemia. Larger studies examining a wider range of clinically available inflammatory biomarkers in a more diverse set of cytokine storm syndromes are warranted.