宿主免疫功能紊乱在发病中起着至关重要的作用,programming,和噬血细胞性淋巴组织细胞增生症（HLH）的结果。本研究旨在综合评价初诊继发性噬血细胞性淋巴组织细胞增生症(sHLH)患者的外周免疫状况,并探讨其对患者预后的预测价值。本研究共纳入77例sHLH患者，其中31人正在经历死亡。流式细胞术用于评估百分比，绝对数,和淋巴细胞亚群的表型。同时,还收集了细胞因子水平和常规实验室指标。在sHLH患者中，淋巴细胞亚群绝对数量显著受损，伴随着T细胞过度激活，B细胞过度激活,和浆细胞增殖增加。预后分析显示，较低的CD8+T细胞百分比，升高的APTT,IL-6，IL-10水平，CD4+CD28nullT细胞比例增加与患者预后不良相关.该研究表明sHLH患者淋巴细胞亚群的计数和表型失调。几个关键因素，包括IL-6，IL-10，APTT，和各种T细胞百分比，有可能作为sHLH的预后标志物和治疗靶标。
Host immune dysfunction plays a crucial role in the onset, progression, and outcome of hemophagocytic lymphohistiocytosis (HLH). This study aimed to comprehensively evaluate the peripheral immune profiles in patients with newly diagnosed secondary hemophagocytic lymphohistiocytosis (sHLH), and explore their predictive value for patient prognosis. A total of 77 patients with sHLH were enrolled in this study, with 31 of them experiencing mortality. Flow cytometry was used to assess the percentages, absolute numbers, and phenotypes of lymphocyte subsets. Simultaneously, cytokine levels and routine laboratory indicators were also collected. In sHLH patients, lymphocyte subset absolute numbers were significantly impaired, accompanied by T cell hyperactivation, B cell hyperactivation, and increased plasmablast proliferation. Prognostic analysis revealed that lower CD8+ T cell percentages, elevated APTT, IL-6, IL-10 levels, and increased CD4+CD28null T cell proportions were associated with poor patient outcomes. The study demonstrates dysregulation in the counts and phenotypes of lymphocyte subsets in sHLH patients. Several key factors, including IL-6, IL-10, APTT, and various T cell percentages, have potential as prognostic markers and therapeutic targets in sHLH.
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