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Treatment of an HLH-mimic disease based on HAVCR2 variants with absent TIM-3 expression

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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.

Keywords: mimic disease; disease based; havcr2 variants; treatment hlh; based havcr2; hlh mimic

Journal Title: Blood Advances
Year Published: 2022

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