An unusual presentation of Hemophagocytic Lymphohistiocytosis – A rare but potentially fatal entity

• Author Details:   
• Ragul Dharmalingam ^*
• Saravin Nallaiyan
• Muralidaran Srinivasan
• Karthikeyan Chenniyappan

Hemophagocytic lymphohistiocytosis (HLH) is an autoimmune phenomenon characterized by reactive hyperactivity of cytotoxic T cells and histiocytes, leading to hypercytokinemic injury to cells and organ system, which leads to multiorgan dysfunction and ultimate failure. We had a patient 26 year old female, with no comorbidities came with very late presentation of psoas abscess (trauma related). On presentation she was encephalopathic hence she got mechanically ventilated. Her lab investigations showed bicytopenia, coagulopathy, hyperbilirubinemia (indirect) with mild transaminits and normal renal parameters. She was started on empirical antibiotics and source control was done. Despite adequate source control, she continued to deteriorate. Pus culture showed E.coli and negative for TB. On extensive evaluation, she was found to have dengue IgM positive. Through out the course she was normothermic. On further evaluation on Day 5, she was found to have normal LDH levels, hypofibrinogenemia and hypertriglyceridemia. Imaging done showed hepatomegaly with normal sized spleen. On day 6, she started to develop bilateral lowerlobe consolidations. On suspicion of HLH, we did bone marrow aspiration that was consistent with hemophagocytosis. We planned to start her on Dexamethasone, but she unfortunately succumbed to death due to underlying sepsis and multiorgan failure. Early suspicion of HLH didn’t arise because of normothermia, very mild transaminits, hyperbilirubinemia and normal sized spleen. Thus, clinical presentation of HLH is variable and early suspicion of this life threatening condition is warranted.