Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of extreme, systemic immune activation characterized by prolonged fever, hepatosplenomegaly, cytopenias, and elevated markers of inflammation. This constellation of clinical and laboratory findings results from dysregulation of the pathways that govern the immune system ’s inflammatory response, leading to persistent activation of lymphocytes and macrophages. The mortality rate is high, and prompt recognition and treatment initiation are essential for survival. However, diagnosis can be challenging because HLH is rare and can present with nonspecific clinical fin dings that overlap with other disease states, such as sepsis or malignancy.
Source: Pediatrics in Review - Category: Pediatrics Source Type: research