title: Hemophagocytic lymphohistiocytosis associated with viral infections: Diagnostic challenges and therapeutic dilemmas.
abstract: Hemophagocytic lymphohistiocytosis is a frequently fatal clinicopathologic syndrome in which an uncontrolled and ineffective immune response leads to severe hyperinflammation. It may occur as either a familial disorder or a sporadic condition in association with a variety of triggers: infections, malignancies, autoimmune diseases, and acquired immune deficiencies. However, the most consistent association is with viral infections, especially Epstein-Barr virus. The main clinical features are fever, liver dysfunction, coagulation abnormalities and pancytopenia. Early diagnosis and treatment are important to reducing mortality, but the diagnosis is difficult because of the rarity of the syndrome and the lack of specificity of the clinical findings. Treatment should be directed towards treating the underlying disease and to suppressing the exaggerated inflammatory response through the use of immunosuppressive agents.
authors.affiliation: Servicio de Medicina Interna, Complejo Asistencial Universitario de León, León, España. Electronic address: jlmostaza@yahoo.es.
authors.forename: J L
authors.initials: JL
authors.lastname: Mostaza-Fernández
authors.affiliation: Servicio de Medicina Interna, Complejo Asistencial Universitario de León, León, España.
authors.forename: J
authors.initials: J
authors.lastname: Guerra Laso
authors.affiliation: Servicio de UCI, Complejo Asistencial Universitario de León, León, España.
authors.forename: D
authors.initials: D
authors.lastname: Carriedo Ule
authors.affiliation: Sección de Inmunología Clínica. Complejo Asistencial Universitario de León, León, España.
authors.forename: J M G
authors.initials: JM
authors.lastname: Ruiz de Morales
keywords: Hemofagocitosis, Infecciones virales; Hemophagocytic lymphohistiocytosis; Hemophagocytosis; Linfohistiocitosis hemofagocítica; Perforin; Perforina; Viral infections