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The innate immune system launches a generalized response to invading organisms, and the adaptive immune system produces specific antibodies to fight those organisms. MAS is caused by an overactive innate immune system. Infections are fought by a number of types of immune cells working together in a coordinated manner. Macrophages attack viruses and bacteria, while T-lymphocytes send signals to gather macrophages and other immune cells. Unfortunately, the immune system is unable to eradicate infectious cells and instead floods the body with inflammatory cytokines, which damage any organ.
Several studies have shown that HLH is associated with MAS, but a recent large survey showed that it occurs in around one-third of cases. The study did not identify a causal role, but noted that pHLH mutations may result in an increased risk of developing the disease. Moreover, Learn More of the patient is an important contributor to MAS. In addition to being viral, MAS episodes typically occur at the onset of the disease.
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