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
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container_start_page 123
container_title Journal of blood medicine
container_volume 15
creator 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
description 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.
doi_str_mv 10.2147/JBM.S436720
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title Case Study: Rosai-Dorfman Disease and Its Multifaceted Aspects
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