An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an immune related clinical syndrome with protean manifestations, varying presentation, clinically complex, with diverse causes, and is an under-recognized entity which carries high morbidity and mortality. It is precipitated by an immunological trigger in a susceptible host resulting in immune activation and dysregulation leading to disruption of immune homeostasis, cytokine storm and multi-organ failure. We describe a case of Epstein-Barr virus (EBV) associated HLH with its typical diagnostic challenges and associated high mortality rate. Certain diagnostic criteria and online tools may help to arrive at an earlier presumptive diagnosis which, in turn, may expedite treatment and lead to better clinical outcomes.

Hemophagocytic Lymphohistiocytosis in a Patient with Hodgkin Lymphoma, HIV, and Epstein-Barr Virus

“…The mortality rate remains high in patients with HLH. In one case series that included 162 patients, the mortality rate reached 43% with treatment, with a higher mortality rate observed among patients with hematologic malignancy [ 10 , 11 ]. It is important to note that early diagnosis and prompt treatment are critical in improving the chances of survival for patients with HLH, as the mortality rate can reach 50%-70% if left untreated [ 7 ].…”