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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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Your Question

What is the best treatment for Rosai-Dorfman? A biopsy was done about 2 years ago. The doctor is considering radiation therapy. I have a lesion deep in my pelvic bone.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

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