Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a multisystem disorder mediated by cytokine storm and is characterized by fever, pancytopenia and organomegaly with laboratory features like hyperferritinemia, hypertriglyceridemia and hypofibrinogenemia1 . Etiology can be genetic or acquired such as infections, malignancy and autoimmune disorders. Diagnosis, identification of underlying etiology and management of HLH remain tough clinical puzzles to sort out for the managing physician. The term hemophagocytosis describes the pathologic finding of activated macrophages, engulfing erythrocytes, leukocytes, platelets and their precursor cells. It is a serious and potentially life threatening histiocytic disorder in children and adults. A hallmark of HLH is impaired or absent function of natural killer (NK) cells and cytotoxic T-cells (CTL)2 . In its most severe form, it leads to a sepsis-like picture and multiorgan failure (MOF).We reported a case of a ten-year-old boy who had such a presentation and succumbed during the hospital stay.