title: Hemophagocytic lymphohistiocytosis (HLH): A heterogeneous spectrum of cytokine-driven immune disorders.
abstract: 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.
authors.affiliation: Laboratory of Immunobiology, Rega Institute, University of Leuven, Minderbroedersstraat 10, B-3000 Leuven, Belgium. Electronic address: ellen.brisse@rega.kuleuven.be.
authors.forename: Ellen
authors.initials: E
authors.lastname: Brisse
authors.affiliation: Laboratory of Pediatric Immunology, University Hospital Gasthuisberg, Herestraat 49, Box 7003, B-3000 Leuven, Belgium. Electronic address: carine.wouters@uz.kuleuven.ac.be.
authors.forename: Carine H
authors.initials: CH
authors.lastname: Wouters
authors.affiliation: Laboratory of Immunobiology, Rega Institute, University of Leuven, Minderbroedersstraat 10, B-3000 Leuven, Belgium. Electronic address: patrick.matthys@rega.kuleuven.be.
authors.forename: Patrick
authors.initials: P
authors.lastname: Matthys
mesh_terms: D000818:Animals; D016207:Cytokines / Q000276:immunology*; D004195:Disease Models, Animal; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy / Q000276:immunology*
chemical_list: D016207:Cytokines
keywords: Animal models; Cytokine storm; Hemophagocytic lymphohistiocytosis; Macrophage activation syndrome; Therapy