Other Names for this Disease
- Sinus histiocytosis with massive lymphadenopathy
- Rosaï-Dorfman disease
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 It is a benign disease which is characterized by over-production and accumulation of a specific type of white blood cell (histiocyte) in the lymph nodes of the body, most often those of the neck (cervical). Other lymph node groups may also be involved and, in some cases, abnormal accumulation of histiocytes may occur in other areas of the body (extranodal). The cause of this condition remains unknown, although altered immune responses and infectious agents may play a role. SHML is a self-limited and seldom life-threatening disease which commonly does not require therapy.Rosai-Dorfman disease was first described by Rosai and Dorfman in 1969.
Last updated: 7/22/2013
- Gomes Pinto DC, de Aguiar Vidigal T, de Castro B, dos Dantos BH, de Sousa NJA. Rosai-Dorfman disease in the differential diagnosis of cervical lymphadenopathy. Rev. Bras. Otorrinolaringol. 2008; http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0034-72992008000400025&lng=en&nrm=iso&tlng=en. Accessed 7/22/2013.
- Rosai J. Rosai-Dorfman Disease. National Organization for Rare Disorders (NORD). 2011; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1122/viewAbstract. Accessed 7/22/2013.
- Tebbi CK, Arceci RJ, Loew TW. Histiocytosis. Medscape Reference. April 2012; http://emedicine.medscape.com/article/958026-overview. Accessed 7/22/2013.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
- The Doctor's Doctor provides information about Rosai-Dorfman disease. Click on the link above to access this information.
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Rosai-Dorfman disease. Click on the link to view a sample search on this topic.