Abstract：
Hemophagocytic lymphohistiocytosis (HLH) comprises a broad spectrum of life-threatening cytokine storm syndromes, classified into primary (genetic) or secondary (acquired) HLH. The latter occurs in a variety of medical conditions, including infections, malignancies, autoimmune and autoinflammatory diseases, acquired immunodeficiency, and metabolic disorders. Despite recent advances in the field, the pathogenesis of secondary HLH remains incompletely understood. Considering the heterogeneity of triggering factors and underlying diseases in secondary HLH, a large diversity of animal models has been developed to explore pivotal disease mechanisms. To date, over 20 animal models have been described that each recapitulates certain aspects of secondary HLH. This review provides a comprehensive overview of the existing models, highlighting relevant findings, discussing the involvement of different cell types and cytokines in disease development and progression, and considering points of interest toward future therapeutic strategies.
摘要：
噬血细胞淋巴组织细胞增生症（HLH）包括广泛的威胁生命的细胞因子风暴综合征，分为原发性（遗传）或继发性（获得性）HLH。后者发生在各种医疗条件下，包括感染,恶性肿瘤,自身免疫性和自身炎症性疾病，获得性免疫缺陷,和代谢紊乱。尽管该领域最近取得了进展，继发性HLH的发病机制尚不完全清楚。考虑到继发性HLH的触发因素和基础疾病的异质性，已经开发了大量的动物模型来探索关键的疾病机制。迄今为止,已经描述了超过20个动物模型,每个都概括了继发性HLH的某些方面。这篇综述全面概述了现有的模型，强调相关发现，讨论不同细胞类型和细胞因子在疾病发展和进展中的参与，并考虑未来治疗策略的兴趣点。
