Article
Genetic defects in cytolysis in macrophage activation syndrome.
Current rheumatology reports - 1 Jan 2014
Zhang Mingce, Behrens Edward M, Atkinson T Prescott, Shakoory Bita, Grom Alexei A, Cron Randy Q
Abstract excerpt
Macrophage activation syndrome (MAS), typically presenting beyond the first year of life, is an often lethal cousin of familial hemophagocytic lymphohistiocytosis (fHLH). Defects in natural killer (NK) cell and CD8 T cell cytotoxicity result in a pro-inflammatory cytokine storm, cytopenia, coagulopathy, and multi-organ system dysfunction. MAS can occur in association with infections (herpes viruses), cancer...
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