title: Acute lupus hemophagocytic syndrome: report of a case and review of the literature.
abstract: The authors reported a case of systemic lupus erythematosus (SLE) with an unusual presentation. The patient presented with acute febrile illness along with progressive pancytopenia related to increasing hemophagocytic activity of histiocytes in the bone marrow. Concomitant polyarthritis, myositis, nephritis, high titer of antinuclear factor (1:2,560) and positive test for anti-DNA antibody made him fit the diagnostic criteria of SLE. No definite evidence of associated infections was confirmed by bacteriologic, serologic and viral studies. He did not respond to empiric antibiotic therapy but dramatically responded to corticosteroid treatment. Therefore, diagnosis of acute lupus hemophagocytic syndrome was made. The clinical presentation, laboratory diagnosis, and management of the patient are discussed and the literature was reviewed and presented.
authors.affiliation: Department of Internal Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand.
authors.forename: Piroon
authors.initials: P
authors.lastname: Mootsikapun
authors.forename: Chittima
authors.initials: C
authors.lastname: Sirijerachai
authors.forename: Kanchana
authors.initials: K
authors.lastname: Chansung
authors.forename: Ratanavadee
authors.initials: R
authors.lastname: Nanagara
mesh_terms: D000208:Acute Disease; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D008297:Male; D010198:Pancytopenia / Q000188:drug therapy / Q000209:etiology / Q000276:immunology; D010587:Phagocytosis / Q000276:immunology; D011239:Prednisolone / Q000627:therapeutic use
chemical_list: D000893:Anti-Inflammatory Agents; D011239:Prednisolone