Hemophagocytic Lymphohistiocytosis in Adults

Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome with established diagnostic criteria, and well characterized gene mutations in children. In contrast, acquired HLH in adults (aHLH) due to the heterogeneity of trigger diseases (infections, malignancies and autoimmune diseases) is less clear defined. There is both a risk of under- and overtreatment. Due to the lack of validated diagnostic criteria for HLH in adults, diagnostic and therapeutic delay are significant risk factors for patient outcome. This article presents the current status and new developments in epidemiology, pathophysiology, diagnosis and treatment of aHLH.