Case Study: Rosai-Dorfman Disease and Its Multifaceted Aspects

Rosai-Dorfman Disease (RDD) is a rare non-Langerhans histiocytosis, usually self-limited and presenting with massive, painless, bilateral cervical lymphadenopathy, with or without constitutional symptoms. Extranodal disease is frequently present, and may happen in the absence of lymph node involveme...

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Published inJournal of blood medicine Vol. 15; pp. 123 - 128
Main Authors Werneck Rodrigues, Daniela Oliveira, Wolp Diniz, Roberta, Dentz, Leonardo Cunha, Costa, Monica de Albuquerque, Lopes, Roberto Heleno, Suassuna, Lucas Fernandes, Cintra, Jane Rocha Duarte, Domenge, Christian
Format Journal Article
LanguageEnglish
Published New Zealand Dove Medical Press Limited 31.03.2024
Dove Medical Press
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Summary:Rosai-Dorfman Disease (RDD) is a rare non-Langerhans histiocytosis, usually self-limited and presenting with massive, painless, bilateral cervical lymphadenopathy, with or without constitutional symptoms. Extranodal disease is frequently present, and may happen in the absence of lymph node involvement, symptomatology and differential diagnosis will depend on the site affected and fatal cases may occur. The authors present two cases of Rosai-Dorfman disease (RDD), diagnosed through immunohistochemistry, with different progressions, one with complete remission and one culminating in death, highlighting the variety of presentations and the diagnostic difficulty. RDD is a rare condition with clinical presentations similar to several diseases, and should be considered in the differential diagnosis of lymphadenopathy with extranodal lesions.
ISSN:1179-2736
1179-2736
DOI:10.2147/JBM.S436720