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Diseases

Genetic and Rare Diseases Information Center (GARD)

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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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Overview



What is Rosai-Dorfman disease?

What are the signs and symptoms of Rosai-Dorfman disease?

What causes Rosai-Dorfman disease?

How might Rosai-Dorfman disease be treated?


What is Rosai-Dorfman disease?

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

What are the signs and symptoms of Rosai-Dorfman disease?

The symptoms and physical findings associated with Rosai-Dorfman disease vary depending upon the specific areas of the body that are affected.[2] Systemic symptoms, such as fever, weight loss, malaise, joint pain, and night sweats, may be present. Cervical lymph nodes are most characteristically involved, but other areas, including extranodal regions, can be affected.[3] These areas include the skin, central nervous system, kidney, and digestive tract.[2] 

Last updated: 7/22/2013

What causes Rosai-Dorfman disease?

The exact cause of Rosai-Dorfman disease is unknown.[1][2] Possible causes include altered immune responses and infections by agents such as varicella-zoster and other herpetic viruses; Epstein-Barr; cytomegalovirus; Brucella; and Klebsiella.[1]
Last updated: 7/22/2013

How might Rosai-Dorfman disease be treated?

In many cases, the signs and symptoms of Rosai-Dorfman disease go away without any treatment (spontaneous remission) within months or a few years. When possible, the preferred course of management is continuous observation without treatment. Many individuals will not require therapy, but in some cases, various treatment options may become necessary. Treatment of the condition is then typically directed toward the specific symptoms that the affected individual has. Some individuals may need surgical removal of histiocytic lesions.[4] In more serious cases, treatment options have included therapy with certain drugs including steroids (e.g., prednisone), alfa-interferon (a man-made copy of a protein that is normally made by the body in response to infection), chemotherapy and radiation therapy.[3][4] These treatments have improved symptoms in some individuals, but in others they have not been effective. Other treatment is symptomatic and supportive.[4]
Last updated: 8/20/2013

References
  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; http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0034-72992008000400025&lng=en&nrm=iso&tlng=en. Accessed 7/22/2013.
  2. 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.
  3. Tebbi CK, Arceci RJ, Loew TW. Histiocytosis. Medscape Reference. April 2012; http://emedicine.medscape.com/article/958026-overview. Accessed 7/22/2013.
  4. Rosai-Dorfman Disease. NORD. April 18, 2011; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1122/viewAbstract. Accessed 12/20/2011.