Ontology highlight
ABSTRACT:
SUBMITTER: Behrens EM
PROVIDER: S-EPMC3104738 | biostudies-literature | 2011 Jun
REPOSITORIES: biostudies-literature
Behrens Edward M EM Canna Scott W SW Slade Katharine K Rao Sheila S Kreiger Portia A PA Paessler Michele M Kambayashi Taku T Koretzky Gary A GA
The Journal of clinical investigation 20110516 6
Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are 2 similar diseases characterized by a cytokine storm, overwhelming inflammation, multiorgan dysfunction, and death. Animal models of HLH suggest that disease is driven by IFN-γ produced by CD8⁺ lymphocytes stimulated by persistent antigen exposure. In these models and patients with "primary" HLH, the antigen persists due to genetic defects, resulting in ineffective cytotoxic responses by CD8⁺ T cells and poor p ...[more]