title: [Adult-onset Still's disease accompanied by hemophagocytic syndrome at onset].
abstract: A 43-year-old woman was admitted for examination of fever, an elevated transaminase level, LDH, skin eruption, sore throat and bicytopenia. As bone marrow examination revealed an increased proportion of histiocytes and active phagocytosis, hemophagocytic syndrome (HPS) was diagnosed. After admission, the peripheral blood counts recovered spontaneously and the HPS subsided, but other symptoms persisted and the neutrophil count increased. At this time, we diagnosed the patient as having adult Still's disease. All the symptoms disappeared after administration of prednisolone. The markedly increased concentrations of TNF-alpha and IFN-gamma in the peripheral blood at the time of HPS declined gradually, and the IL-6 concentration increased at the time of diagnosis of Still's disease. However, all of these concentrations normalized after administration of prednisolone. As HPS and Still's disease have a common etiology, and each shows high concentrations of IFN-gamma, IL 6 and TNF-alpha, the symptoms are similar in both diseases. In particular, a relationship between HPS and high concentrations of TNF-alpha and IFN-gamma is suspected.
authors.affiliation: Internal Medicine, Higashi Sapporo Hospital.
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Hirayama
authors.forename: Sumio
authors.initials: S
authors.lastname: Sakamaki
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Tsuji
authors.forename: Tamotsu
authors.initials: T
authors.lastname: Sagawa
authors.forename: Takaharu
authors.initials: T
authors.lastname: Nakajima
authors.forename: Takuya
authors.initials: T
authors.lastname: Matsunaga
authors.forename: Katsuhisa
authors.initials: K
authors.lastname: Kogawa
authors.forename: Junji
authors.initials: J
authors.lastname: Kato
authors.forename: Yoshiro
authors.initials: Y
authors.lastname: Niitsu
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D016207:Cytokines / Q000097:blood; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D011239:Prednisolone / Q000008:administration & dosage; D016706:Still's Disease, Adult-Onset / Q000150:complications*
chemical_list: D000893:Anti-Inflammatory Agents; D016207:Cytokines; D011239:Prednisolone