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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Veröffentlicht in:Journal of blood medicine 2024, Vol.15, p.123-128
Hauptverfasser: 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: Report
Sprache:eng
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Zusammenfassung: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