title: [Macrophage activation syndrome: a diagnostic challenge].
abstract: Macrophage activation syndrome, or hemophagocytic syndrome, is a rare disease with high morbidity and mortality. It is a disorder of the mononuclear phagocyte system. Two forms have been described; primary or familial hemophagocytic lymphohistiocytosis and secondary or sporadic hemophagocytic syndrome. Diagnosis of macrophage activation syndrome poses a real challenge for the pediatrician and in many cases is only made at autopsy. We describe two patients with hemophagocytic syndrome associated with parvovirus B19 and Epstein-Barr virus infection.
authors.affiliation: Unidad de Cuidados Intensivos Pediátricos, Hospital Infantil Universitario Virgen del Rocío, Sevilla, Spain. jasoult@terra.es
authors.forename: J A
authors.initials: JA
authors.lastname: Soult Rubio
authors.forename: V
authors.initials: V
authors.lastname: García Bernabeu
authors.forename: M J
authors.initials: MJ
authors.lastname: Sánchez Alvarez
authors.forename: M
authors.initials: M
authors.lastname: Muñoz Sáez
authors.forename: J D
authors.initials: JD
authors.lastname: López Castilla
authors.forename: A
authors.initials: A
authors.lastname: Tovaruela Santos
mesh_terms: D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis* / Q000503:physiopathology; D017809:Fatal Outcome; D005260:Female; D005334:Fever; D004854:Herpesvirus 4, Human; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis* / Q000821:virology*; D006801:Humans; D009102:Multiple Organ Failure / Q000209:etiology; D010322:Parvoviridae Infections / Q000175:diagnosis* / Q000503:physiopathology; D016732:Parvovirus B19, Human; D013163:Splenomegaly