title: Erdheim-Chester disease: report of a case with PCR-based analysis of the expression of osteopontin and survivin in Xanthogranulomas following glucocorticoid treatment.
abstract: Erdheim-Chester disease (ECD) is a form of non-Langerhans histiocytosis. In this report, we show a case of ECD presenting diabetes insipidus and multiple xanthogranulomas received glucocorticoid treatment over a year. During this period, xanthogranulomas improved in response to the glucocorticoid therapy. Furthermore, the expression of osteopontin in xanthogranulomatous tissues significantly decreased following the treatment. Our data show the expression of osteopontin in xanthogranulomatous tissues of ECD. Furthermore, the osteopontin mRNA decreased following glucocorticoid therapy with xanthogranuloma regression, suggesting that the expression level of osteopontin could be a marker of the disease activity of ECD.
authors.affiliation: Department of Endocrinology, Kochi Medical School, Kochi University, Nankoku, Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Taguchi
authors.forename: Yasumasa
authors.initials: Y
authors.lastname: Iwasaki
authors.forename: Koichi
authors.initials: K
authors.lastname: Asaba
authors.forename: Toshinori
authors.initials: T
authors.lastname: Yoshida
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Takao
authors.forename: Fuminori
authors.initials: F
authors.lastname: Ikeno
authors.forename: Hideaki
authors.initials: H
authors.lastname: Nakajima
authors.forename: Hajime
authors.initials: H
authors.lastname: Kodama
authors.forename: Kozo
authors.initials: K
authors.lastname: Hashimoto
mesh_terms: D000328:Adult; D031249:Erdheim-Chester Disease / Q000188:drug therapy* / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D015870:Gene Expression / Q000187:drug effects; D005938:Glucocorticoids / Q000627:therapeutic use; D006099:Granuloma / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D051034:Inhibitor of Apoptosis Proteins; D008869:Microtubule-Associated Proteins / Q000235:genetics*; D009363:Neoplasm Proteins / Q000235:genetics*; D053495:Osteopontin / Q000235:genetics*; D016133:Polymerase Chain Reaction; D011239:Prednisolone / Q000627:therapeutic use*; D000077022:Survivin; D014973:Xanthomatosis / Q000188:drug therapy* / Q000235:genetics
chemical_list: C107489:BIRC5 protein, human; D005938:Glucocorticoids; D051034:Inhibitor of Apoptosis Proteins; D008869:Microtubule-Associated Proteins; D009363:Neoplasm Proteins; D000077022:Survivin; D053495:Osteopontin; D011239:Prednisolone