Epstein-Barr virus--Associated hemophagocytic syndrome. A cause of fever of unknown origin in human immunodeficiency virus infection.

A 26-year-old human immunodeficiency virus-positive man presented with fever of unknown origin, pancytopenia, and elevated liver function tests. Numerous diagnostic tests and empiric therapeutic interventions remained unsuccessful. Splenectomy eventually established the diagnosis of Epstein-Barr virus-associated hemophagocytic syndrome. Treatment with foscarnet, acyclovir, prednisone, and vinblastine resulted in complete recovery. Three months later, Hodgkin's disease, a previously reported complication of the Epstein-Barr virus-associated hemophagocytic syndrome, was diagnosed. Fever, pancytopenia, and hepatic dysfunction are common complication of advanced human immunodeficiency virus infection and can be caused by a variety of opportunistic pathogens. A high index of suspicion is critical for the management of this otherwise fatal disorder because once the diagnosis is made, even patients with advanced disease benefit from aggressive therapeutic intervention, as demonstrated in the case presented.