"HLH is a syndrome of excessive inflammation and tissue destruction due to abnormal immune activation. The abnormal immune activation leads to a hyperinflammatory immune state which is thought to be caused by the absence of normal downregulation of macrophages and lymphocytes.

HLH can be classified as primary or secondary. Primary HLH is hereditary and caused by defects in perforin or granzymes B mediated toxicity. Secondary causes of HLH include infections (EBV), malignancy and autoimmune disease."

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