Haemophagocytic lymphohistiocytosis

Abstract

We describe a 15-month-old child who presented with a history of fever for the preeding two days and multiple episodes of seizures of one day duration. Physical examination revealed generalized lymphadenopathy and hepatosplenomegaly. The child developed erythematous maculopapular rash, hypotension and required tracheal intubation and mechanical ventilation. Bone marrow aspiration confirmed the diagnosis of hemophagocytic lymphohistiocytosis (HLH). He was treated with dexamethasone, etoposide and cyclosporine, recovered and is doing well on follow-up. HLH is a rare haematological disorder involving the mononuclear phagocyte system. Since it mimics other disorders, its timely diagnosis remains a challenge. It is an aggressive and potential fatal disease of infancy and childhood, if left untreated. HLH may mimic a number of systemic infections thereby causing difficulty in early diagnosis.