Article
Polygenic mutations in the cytotoxicity pathway increase susceptibility to develop HLH immunopathology in mice.
Blood - 28 Apr 2016
Sepulveda Fernando E, Garrigue Alexandrine, Maschalidi Sophia, Garfa-Traore Meriem, Ménasché Gaël, Fischer Alain, de Saint Basile Geneviève
Abstract excerpt
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory disease. Inherited forms of HLH are caused by biallelic mutations in several effectors of granule-dependent lymphocyte-mediated cytotoxicity. A small proportion of patients with a so-called "secondary" form of HLH, which develops in the aftermath of infection, autoimmunity, or cancer, carry a monoallelic mutation in one or more...
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