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Rosai-Dorfman disease: a report of eight cases in a tertiary care center and a review of the literature 56
Maia,R.C.; de Meis,E.; Romano,S.; Dobbin,J.A.; Klumb,C.E..
Rosai-Dorfman disease (RDD) is a nonmalignant histiocytic disorder of unknown origin that is extremely rare. By immunohistochemistry, the RDD cells are characteristically S-100 positive and CD1a negative. Emperipolesis is a common histopathological finding, although not specific for RDD. Lymph node and cutaneous manifestations are most frequent, but diverse organs can be affected. The clinical course is unpredictable regardless of treatment. Here, we present a series of 8 cases presenting lymph node and/or cutaneous lesions. Lymph node involvement was seen in diverse regions, including mediastinal and retroperitoneal. The treatment response to steroids was diversified, and the chemotherapy response was disappointing. Associated autoimmune diseases (Sjögren...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Rosai-Dorfman disease; Sinus histiocytosis with massive lymphadenopathy; Histiocytosis.
Ano: 2015 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2015000100006
Registros recuperados: 1
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