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Rosai-Dorfman disease

Other Names for this Disease
  • RDD
  • Rosaï-Dorfman disease
  • SHML
  • Sinus histiocytosis with massive lymphadenopathy
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Rosai-Dorfman disease was first described by Rosai and Dorfman in 1969.[1] 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).[2] 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).[1][2] 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.[1][3]
Last updated: 7/22/2013


  1. 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; Accessed 7/22/2013.
  2. Rosai J. Rosai-Dorfman Disease. National Organization for Rare Disorders (NORD). 2011; Accessed 7/22/2013.
  3. Tebbi CK, Arceci RJ, Loew TW. Histiocytosis. Medscape Reference. April 2012; Accessed 7/22/2013.
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Basic Information

  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
  • The Doctor's Doctor provides information about Rosai-Dorfman disease. Click on the link above to access this information.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • 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.