Hematologic Disease Laboratory; Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health 100045, China.
Hematology Center; Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health 100045, China.
Cytokine. 2022 Jan;149:155729. doi: 10.1016/j.cyto.2021.155729. Epub 2021 Oct 19.
Goal of this research was to investigate values of serum cytokines in childhood HLH with different triggers, with the expectation to find secretion spectrum of 5 main types of underlying diseases.
118 newly diagnosed HLH were included, and serum concentrations of 6 cytokines were tested before treatment began. Absolute cytokine levels and ratios between them were then studied in the HLH groups collectively and separately RESULTS: In general, IFN-γ, IL-10 and IL-6 showed differences among 5 HLH groups. Specifically, relative levels of these three cytokines to each other were meaningful in distinguishing 4 types of HLH. Level of IL-6 was higher than those of IFN-γ or IL-10 in HLH driven by Systemic auto-inflammatory disorders (SAIDs) or Langerhans Cell Histiocytosis (LCH), while primary HLH and EBV-HLH shared elevated ratio of IL-10 to IL-6. Although more than one distinctive ratios were found in 3 HLH groups, combination of these parameters didn't offer optimal balance between sensitivity and specificity.
As a group of easily gained laboratory findings, cytokine levels were reliable in the procedure of roughly classifying HLH cases with the help of patients' clinical phenotype. However, adequate data is still needed to explore the significance of these indicators in identifying one particular underlying disease accurately.
本研究旨在探讨不同诱因儿童噬血细胞综合征(HLH)患者血清细胞因子的价值,期望找到 5 种主要潜在疾病的分泌谱。
纳入 118 例新诊断的 HLH 患者,在开始治疗前检测 6 种细胞因子的血清浓度。然后研究 HLH 组中这些细胞因子的绝对水平及其比值。
总体而言,IFN-γ、IL-10 和 IL-6 在 5 个 HLH 组之间存在差异。具体而言,这三种细胞因子之间的相对水平对于区分 4 种 HLH 具有重要意义。由系统性自身炎症性疾病(SAIDs)或朗格汉斯细胞组织细胞增生症(LCH)驱动的 HLH 中,IL-6 水平高于 IFN-γ或 IL-10,而原发性 HLH 和 EBV-HLH 则共同具有升高的 IL-10 与 IL-6 比值。虽然在 3 个 HLH 组中发现了多个独特的比值,但这些参数的组合并不能在敏感性和特异性之间提供最佳平衡。
作为一组易于获得的实验室发现,细胞因子水平在患者临床表型的帮助下对 HLH 病例进行大致分类的过程中是可靠的。然而,仍需要更多数据来探讨这些指标在准确识别特定潜在疾病方面的意义。