Toxic activation of immune cells can lead to life-threatening conditions, all previously named hemophagocytic lymphohistiocytosis (HLH). Increased knowledge of the etiology and various underlying pathologies deems new nomenclature necessary. 1… Click to show full abstract
Toxic activation of immune cells can lead to life-threatening conditions, all previously named hemophagocytic lymphohistiocytosis (HLH). Increased knowledge of the etiology and various underlying pathologies deems new nomenclature necessary. 1 HLH and HLH mimicries are life-threatening diseases characterized by uncontrolled immune hyperactivation. They are caused by overactivation of lymphocytes and mac-rophages producing large amounts of interferon- g , interleukin-1 b (IL-1 b ), and IL-18. Different underlying etiologies, including genetic defects, autoimmunity, infections, and malignancies are increasingly recog-nized, adding to the debate about classi fi cation.
               
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