World Library  
Flag as Inappropriate
Email this Article

Rosai–Dorfman disease

Article Id: WHEBN0014099112
Reproduction Date:

Title: Rosai–Dorfman disease  
Author: World Heritage Encyclopedia
Language: English
Subject: List of diseases (R), List of physicians, Non-Langerhans cell histiocytosis
Collection:
Publisher: World Heritage Encyclopedia
Publication
Date:
 

Rosai–Dorfman disease

Rosai-Dorfman disease
Classification and external resources
10 D76.3 (ILDS D76.360)
DiseasesDB 31419

Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare disorder of unknown etiology that is characterized by abundant histiocytes in the lymph nodes throughout the body.[1]:747

Presentation

Lymphadenopathy of the neck is the most common place of histiocyte accumulation, although accumulation outside of lymph nodes may occur, as well. The most common sites of accumulation outside of the lymph nodes are skin, upper respiratory tract, and the sinuses.[2][3]

The symptoms of this disease vary with the site of accumulation similar to other regional tumors. For instance, accumulation in closed spaces such as the cranium can lead to poor outcomes compared to growth in the dermis of an extremity where surgical excision is possible.

The disease is usually non-progressive and self-limited, but patients can manifest autoimmune hemolytic anemia.It can rarely present with massive hemoptysis (coughing of blood)[4]

History

This condition has been named after Ronald F. Dorfman and Juan Rosai. An alternative eponym of this condition is known as Destombes-Rosai-Dorfman syndrome, part of which is named after Pierre-Paul Louis Lucien Destombes.

See also

References


This article was sourced from Creative Commons Attribution-ShareAlike License; additional terms may apply. World Heritage Encyclopedia content is assembled from numerous content providers, Open Access Publishing, and in compliance with The Fair Access to Science and Technology Research Act (FASTR), Wikimedia Foundation, Inc., Public Library of Science, The Encyclopedia of Life, Open Book Publishers (OBP), PubMed, U.S. National Library of Medicine, National Center for Biotechnology Information, U.S. National Library of Medicine, National Institutes of Health (NIH), U.S. Department of Health & Human Services, and USA.gov, which sources content from all federal, state, local, tribal, and territorial government publication portals (.gov, .mil, .edu). Funding for USA.gov and content contributors is made possible from the U.S. Congress, E-Government Act of 2002.
 
Crowd sourced content that is contributed to World Heritage Encyclopedia is peer reviewed and edited by our editorial staff to ensure quality scholarly research articles.
 
By using this site, you agree to the Terms of Use and Privacy Policy. World Heritage Encyclopedia™ is a registered trademark of the World Public Library Association, a non-profit organization.
 



Copyright © World Library Foundation. All rights reserved. eBooks from World eBook Library are sponsored by the World Library Foundation,
a 501c(4) Member's Support Non-Profit Organization, and is NOT affiliated with any governmental agency or department.