Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
Adv Exp Med Biol. 2024;1448:573-582. doi: 10.1007/978-3-031-59815-9_38.
A vast body of evidence provides support to a central role of exaggerated production of interferon-γ (IFN-γ) in causing hypercytokinemia and signs and symptoms of hemophagocytic lymphohistiocytosis (HLH). In this chapter, we will describe briefly the roles of IFN-γ in innate and adaptive immunity and in host defense, summarize results from animal models of primary HLH and secondary HLH with particular emphasis on targeted therapeutic approaches, review data on biomarkers associated with activation of the IFN-γ pathway, and discuss initial efficacy and safety results of IFN-γ neutralization in humans.
大量证据支持干扰素-γ（IFN-γ）过度产生在引起细胞因子血症和噬血细胞性淋巴组织细胞增生症（HLH）的体征和症状中起核心作用。在本章中，我们将简要描述 IFN-γ在先天和适应性免疫以及宿主防御中的作用，总结原发性 HLH 和继发性 HLH 动物模型的结果，特别强调靶向治疗方法，回顾与 IFN-γ通路激活相关的生物标志物数据，并讨论 IFN-γ中和在人类中的初步疗效和安全性结果。
