Abstract：
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.
摘要：
大量证据支持干扰素-γ（IFN-γ）的过度产生在引起高细胞因子血症以及噬血细胞淋巴组织细胞增多症（HLH）的体征和症状中的核心作用。在这一章中,我们将简要描述IFN-γ在先天和适应性免疫和宿主防御中的作用，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途径激活相关的生物标志物数据，并讨论IFN-γ中和对人体的初步疗效和安全性结果。
