title: [Hemophagocytic syndromes].
abstract: Hemophagocytic syndromes are the clinicobiological translation of an unconnected macrophagic activity with hemophagocytosis. Their physiopathology is related with a deregulation of the T lymphocytes and an excessive production of cytokines. Acquired hemophagocytic syndromes are mostly associated with underlying pathology which they can reveal: immunodeficiency, infections (mostly of viral origin), hemopathies and cancers, auto-immune diseases. The main clinicobiological features are fever, hepatosplenomegaly and peripheric bicytopeny. In the majority of cases, the diagnosis is confirmed by a myelogram which shows the presence of benign histiocytes, actively phagocyting the hematopoietic cells. The pejorative prognosis of hemophagocytic syndromes (actual mortality rate 30 to 45%) requires an early therapy which associates etiological treatment of the underlying affection with pathogenic treatment (pulse of corticosteroids, immunoglobulins, immunosuppressors, or plasmapheresis).
authors.affiliation: Service de médecine interne, HIA Desgenettes, Lyon, France.
authors.forename: A
authors.initials: A
authors.lastname: Fléchaire
authors.forename: B
authors.initials: B
authors.lastname: Colle
authors.forename: P
authors.initials: P
authors.lastname: Bernard
authors.forename: O
authors.initials: O
authors.lastname: Dupuy
authors.forename: P
authors.initials: P
authors.lastname: Philippe
mesh_terms: D000328:Adult; D002648:Child; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D008297:Male; D011379:Prognosis
vernacular_title: Les syndromes hémophagocytaires.