Article

Erdheim-Chester disease: report of a case with PCR-based analysis of the expression of osteopontin and survivin in Xanthogranulomas following glucocorticoid treatment

Endocr J 55:217-23. DOI:JST.JSTAGE/endocrj/K07E-044 [pii] ISBN: 1348-4540 (Electronic) 0918-8959 (Linking) pp.217-23

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.

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Keywords

diabetes insipidus
 
disease activity
 
Erdheim-Chester disease
 
expression level
 
glucocorticoid therapy
 
glucocorticoid treatment
 
multiple xanthogranulomas
 
non-Langerhans histiocytosis
 
osteopontin
 
osteopontin mRNA
 
xanthogranuloma regression
 
xanthogranulomas
 
xanthogranulomatous tissues