A rare complication of infection with the Epstein-Barr virus is the development of
hemophagocytic lymphohistiocytosis. Although most cases of Epstein-Barr virus–induced
hemophagocytic lymphohistiocytosis develop in immunocompetent individuals, the rare
immunodeficiency X-linked lymphoproliferative disease is often unmasked by Epstein-Barr
virus infection and is clinically indistinguishable from Epstein-Barr virus–induced
hemophagocytic lymphohistiocytosis. We describe the clinical course and management of a …