期刊
CYTOKINE & GROWTH FACTOR REVIEWS
卷 26, 期 3, 页码 263-280出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.cytogfr.2014.10.001
关键词
Hemophagocytic lymphohistiocytosis; Macrophage activation syndrome; Cytokine storm; Animal models; Therapy
资金
- Agency for Innovation by Science and Technology (IWT)
- Regional Government of Flanders (GOA program)
- Fund for Scientific Research Flanders (FWO-Vlaanderen)
- Interuniversity Attraction Poles (TAP)
- IWT
Hemophagocytic lymphohistiocytosis (HLH) comprises a group of life-threatening immune disorders classified into primary or secondary HLH. The former is caused by mutations in genes involved in granule-mediated cytotoxicity, the latter occurs in a context of infections, malignancies or autoimmune/autoinflammatory disorders. Both are characterized by systemic inflammation, severe cytokine storms and immune-mediated organ damage. Despite recent advances, the pathogenesis of HLH remains incompletely understood. Animal models resembling different subtypes of HLH are therefore of great value to study this disease and to uncover novel treatment strategies. In this review, all known animal models of HLH will be discussed, highlighting findings on cell types, cytokines and signaling pathways involved in disease pathogenesis and extrapolating therapeutic implications for the human situation. (C) 2014 Elsevier Ltd. All rights reserved.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据