Jun

24

2022

What is the pathogenesis of hemophagocytic lymphohistiocytosis (HLH)?

By William Aird

Caused by congenital or acquired immune dysregulation involving defects (inherited or acquired) in cytotoxic pathways of natural killer (NK) cells and/or cytotoxic T lymphocytes, leading to hypercytokinemia and an accumulation of activated lymphocytes and macrophages (termed histiocytes) in organs and tissues.