title: [Hemophagocytic syndrome in HIV infection].
abstract: This report describes the clinical spectrum and outcome of the hemophagocytic syndrome (HS) in 5 HIV infected patients. All 5 patients presented with fever, hepatomegaly and/or splenomegaly, confusion or coma and respiratory symptoms. Severe anemia was associated with thrombocytopenia and with neutropenia in 4 cases. Diffuse intravascular coagulopathy was present in 2 cases. Liver function tests were abnormal in three patients. The diagnosis of HS was made 2 to 12 weeks after the onset of symptoms and required in most patients repeated examinations of the bone-marrow, showing infiltration by histiocytes with prominent phagocytosis of blood cells. In one case this infiltration was not seen in the bone-marrow but only in the liver and the spleen. Varicella, mycobacterium infection, oesophageal candidiasis, Kaposi sarcoma were observed in the evolution of 3 patients. Anaplastic large cell Ki-1 lymphoma was present in one case. Four patients died as a result of complications of HS. The one patient with lymphoma survived.
authors.affiliation: Service d'Immuno-Hématologie, Hôpital Saint-Louis, Paris.
authors.forename: P
authors.initials: P
authors.lastname: Bourquelot
authors.forename: E
authors.initials: E
authors.lastname: Oksenhendler
authors.forename: M
authors.initials: M
authors.lastname: Wolff
authors.forename: S
authors.initials: S
authors.lastname: Fegueux
authors.forename: C
authors.initials: C
authors.lastname: Piketty
authors.forename: M F
authors.initials: MF
authors.lastname: D'Agay
authors.forename: J P
authors.initials: JP
authors.lastname: Clauvel
mesh_terms: D000328:Adult; D001803:Blood Transfusion; D005260:Female; D015658:HIV Infections / Q000097:blood / Q000150:complications*; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood / Q000209:etiology* / Q000628:therapy; D006801:Humans; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D013156:Splenectomy
chemical_list: D011241:Prednisone
vernacular_title: Syndrome d'hémophagocytose au cours de l'infection par le VIH.