Erdheim-Chester Disease: Report of a Case with PCR-based Analysis of the Expression of Osteopontin and Survivin in Xanthogranulomas Following Glucocorticoid Treatment

• Published in: Endocrine Journal Vol. 55; no. 1; pp. 217 - 223
• Main Authors: TAGUCHI, Takafumi, IWASAKI, Yasumasa, ASABA, Koichi, YOSHIDA, Toshinori, TAKAO, Toshihiro, IKENO, Fuminori, NAKAJIMA, Hideaki, KODAMA, Hajime, HASHIMOTO, Kozo
• Format: Journal Article
• Language: English
• Published: Japan The Japan Endocrine Society 01.01.2008
• Subjects: Adult; Diabetes insipidus; Erdheim-Chester disease; Erdheim-Chester Disease - drug therapy; Erdheim-Chester Disease - genetics; Female; Follow-Up Studies; Gene Expression - drug effects; Glucocorticoids - therapeutic use; Granuloma - drug therapy; Granuloma - genetics; Humans; Inhibitor of Apoptosis Proteins; Microtubule-Associated Proteins - genetics; Neoplasm Proteins - genetics; Non-Langerhans histiocytosis; Osteopontin; Osteopontin - genetics; Polymerase Chain Reaction; Prednisolone - therapeutic use; Survivin; Xanthogranuloma; Xanthomatosis - drug therapy; Xanthomatosis - genetics
• ISSN: 0918-8959; 1348-4540
• DOI: 10.1507/endocrj.K07E-044

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.