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The term of macrophage activation syndrome (MAS) refers to a condition caused by excessive activation and expansion of T lymphocytes and macrophagic histiocytes that exhibit hemophagocytic activity. The expansion of these cells also leads to a massive systemic inflammatory response associated with pan cytopenia, liver dysfunction, and coagulopathy consistent by disseminated intravascular coagulation (DIC).  MAS has been reported in association with almost any rheumatic disease and most common in systemic onset JIA. However, the pathological mechanisms of MAS are not fully understood.

pediatric rheumatology online journal

Effect of listeriolysin O (LLO) secreted by listeria monocytogenes on apoptosis of macrophages derived from patients with macrophage activation syndrome- in vitro