title: [Liver involvement in hemophagocytic syndrome].
abstract: Hemophagocytic syndrome (HPS), whether familial or acquired, is a clinico-pathological entity, characterized by uncontrolled hyper-inflammation, leading sometimes to a lifethreatening condition. Liver dysfunction is central in HPS: hepatomegaly is discovered in half cases, liver enzyme elevation and/or cholestasis are very frequent, cholestasis is a prognostic factor, and liver biopsy is often necessary to confirm HPS and to diagnose the underlying disorders in secondary HPS. The spectrum is large from acquired SAM than can be cured with the treatment of his trigger, to genetic cases only cured by stem cell transplantation.
authors.affiliation: Service de médecine interne, APHP, Hôpital Avicenne, Bobigny Cedex. claire.larroche@avc.aphp.fr
authors.forename: Claire
authors.initials: C
authors.lastname: Larroche
authors.forename: Marianne
authors.initials: M
authors.lastname: Ziol
authors.forename: Sémia
authors.initials: S
authors.lastname: Zidi
authors.forename: Robin
authors.initials: R
authors.lastname: Dhote
authors.forename: Dominique
authors.initials: D
authors.lastname: Roulot
mesh_terms: D015703:Antigens, CD / Q000097:blood; D015214:Antigens, Differentiation, Myelomonocytic / Q000097:blood; D016207:Cytokines / Q000097:blood; D006801:Humans; D008107:Liver Diseases / Q000150:complications*; D008111:Liver Function Tests; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D011379:Prognosis; D011956:Receptors, Cell Surface / Q000097:blood; D000098902:CD163 Antigen
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D000098902:CD163 Antigen; D016207:Cytokines; D011956:Receptors, Cell Surface