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Lessons Learnt in a 17 Year Old with Fever of Unknown Origin: Haemophagocytic Lymphohistiocytosis-The Most Fatal Outcome of EBV Infection

Hajir Ibraheim, Kawai Yip and Siamak Arami

We report a young patient who had persistent fever as a result of the most fatal outcome of an Epstein-Barr Virus infection-haemophagocytic lymphohistiocytosis (EBV-HLH). A 17 year old girl with a background of IgG subclass deficiency presented with a history of fever, sore throat and vomiting. The impression was of EBV related infectious mononucleosis which was supported by a positive monospottest and high EBV DNA levels. Despite a decreasing level of EBV DNA levels, she remained febrile and tachycardic with worsening liver function, cytopenia and no improvement on broad spectrum antimicrobials. Repeated septic screens were negative.
By day 16 HLH was considered and a bone marrow aspirate showed haemphagocytosis. She was started on steroids with a rapid clinical and biochemical response. This case highlights the importance of having a low threshold of suspicion for EBV-HLH in patients with EBV that continue to deteriorate, particularly with a history of immunodeficiency.