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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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Treatment


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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.[1] 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.[2][1] These treatments have improved symptoms in some individuals, but in others they have not been effective. Other treatment is symptomatic and supportive.[1]
Last updated: 8/20/2013

References
  1. Rosai-Dorfman Disease. NORD. April 18, 2011; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1122/viewAbstract. Accessed 12/20/2011.
  2. Tebbi CK, Arceci RJ, Loew TW. Histiocytosis. Medscape Reference. April 2012; http://emedicine.medscape.com/article/958026-overview. Accessed 7/22/2013.
  3. 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.