Hemophagocytic syndrome (HPS) or hemophagocytic lymphohistiocytosis (HLH) is an acute and rapidly progressive systemic inflammatory disorder characterized by cytopenia, excessive cytokine production and hyperferritinemia. Common clinical manifestations of HLH are acute unremitting fever, lymphadenopathy, hepatosplenomegaly and multiple-organ failure. It consists of two different conditions that may be difficult to distinguish: a primary form is mostly seen in children and is caused by various mutations with genetic inheritance and a secondary HLH may be secondary to a malignant, infectious, or autoimmune/autoinflammatory […]
