Many biomarkers have been proposed for the diagnosis of secondary hemophagocytic lymphohistiocytosis (HLH) in adults, but comparative studies are lacking. We analyzed ferritin, glycosylated ferritin, soluble CD25, CD163 and CD14, IL-6, IFN-γ, IL-18, IL-10, IL-1ß, IL-12p70, IL-17α, IP-10, and CXCL9 levels to differentiate HLH from sepsis in critically ill patients. Of 120 patients, HLH was confirmed for 14 patients. Among the biomarkers tested, ferritin, IL-18, and glycosylated ferritin were the most efficient parameters for early diagnosis of HLH. With a sensitivity set at 85%, ferritin, IL-18, and glycosylated ferritin were the biomarkers with the highest specificity: 84, 79, and 71% respectively. Combining IL-18 with the HScore provided a new score with an increased specificity compared to the HScore alone, 86% compared to 70% with a sensitivity set at 100%. A distinct cytokine pattern was highlighted in patients with malignancy-triggered HLH, with highly increased levels of INF-ɣ and CXCL9, compared to HLH secondary to infection. This is the largest study available to date, comparing diagnostic biomarkers for HLH on a cohort of critically ill adult patients. Serum ferritin was the most discriminating parameter for early diagnosis of secondary HLH. IL18^*HScore was identified as a highly potential score.

许多生物标志物已被提出用于诊断成人继发性噬血细胞性淋巴组织细胞增多症 (HLH)，但缺乏比较研究。我们分析了铁蛋白、糖基化铁蛋白、可溶性 CD25、CD163 和 CD14、IL-6、IFN-γ、IL-18、IL-10、IL-1ß、IL-12p70、IL-17α、IP-10 和 CXCL9 水平区分危重患者的 HLH 和脓毒症。在 120 名患者中，14 名患者确诊为 HLH。在测试的生物标志物中，铁蛋白、IL-18 和糖基化铁蛋白是早期诊断 HLH 最有效的参数。在灵敏度设置为 85% 的情况下，铁蛋白、IL-18 和糖基化铁蛋白是特异性最高的生物标志物：分别为 84%、79% 和 71%。与单独的 HScore 相比，将 IL-18 与 HScore 结合提供了具有更高特异性的新分数，86% 与 70% 相比，灵敏度设置为 100%。与继发于感染的 HLH 相比，在恶性肿瘤引发的 HLH 患者中突出显示了一种独特的细胞因子模式，其 INF-ɣ 和 CXCL9 水平显着升高。这是迄今为止最大的研究，比较了一组危重成人患者的 HLH 诊断生物标志物。血清铁蛋白是早期诊断继发性 HLH 最具鉴别性的参数。IL18 血清铁蛋白是早期诊断继发性 HLH 最具鉴别性的参数。IL18 血清铁蛋白是早期诊断继发性 HLH 最具鉴别性的参数。IL18^* HScore 被确定为极具潜力的分数。