Diagnosis and treatment of hemophagocytic lymphohistiocytosis in an adult patient with ehrlichiosis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and often fatal disease, characterized by uninhibited monocyte activation and tissue destruction from an immense inflammatory response. Most cases have been described in the pediatric population, though a rising number of cases have been identified in adults. Adult cases are typically secondary to a triggering process, of which infectious diseases such as Ehrlichia have been implicated. Our patient was an adult male who presented with multi-system organ failure secondary to HLH in the setting of documented Ehrlichia infection, with probable Epstein-Barr Virus (EBV) reactivation. His critical illness resolved after starting appropriate HLH therapy and specific treatment directed at Ehrlichia. This case demonstrates the importance of early recognition and treatment of HLH and its associated triggers in adult patients.