Hemophagocytic lymphohistiocytosis is an unusual syndrome characterized by fever, splenomegaly, jaundice and the pathological finding of hemophagocytosis (phagocytosis by the macrophages of erythrocytes, leucocytes, platelets and their precursors) in bone marrow and other tissues.HLH may be diagnosed in association with malignant, genetic or autoimmune diseases but is also predominantly linked with Ebstein-Barr Virus (EBV) infection. Hyperproduction of cytokines including interferon gamma and tumour necrosis factor-alpha by EBV infected T lymphocytes may play a role in the pathogenesis of HLH. Here we present a young female who presented with HLH associated with EBV infection. The rapid deterioration of clinical condition with multisystem involvement mandates a strong suspicion for the possibility of HLH. Such a clinical scenario in the presence of high ferritin and triglycerides with low fibrinogen helps in timely diagnosis and prompt initiation of immunosuppressant therapy; thereby improving the outcome of a fatal disease.
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