Hemophagocytic Lymphohistiocytosis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a disease characterised by pathological immune activation, fatal hyperinflammation, tissue injury and death if left untreated. It may be primary (genetic or familial) due to mutation in the genes involved in granule dependent cytolytic secretory pathway or sporadic, associated with a variety of triggers. The disease is characterized by fever, cytopenias, splenomegaly, hypertriglyceridemia and/or hypofibrinogenemia and hemophagocytosis. The clinical picture may be mimicked by many conditions like infections, systemic inflammatory response syndrome, rheumatological diseases, malignancies etc, causing the timely diagnosis a challenge. Early recognition and institution of chemoimmunotherapy improves the chance of survival in this potentially fatal disease.