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Rosai-Dorfman Disease

Abstract

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NORD is very grateful to Juan Rosai, MD, Pathology Cancer Center Clinics, Italian Diagnostic Center (CDI), Milan, Italy for assistance in the preparation of this report.

Synonyms of Rosai-Dorfman Disease

  • RDD
  • SHML
  • sinus histiocytosis with massive lymphadenopathy

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Rosai-Dorfman disease is a rare disorder characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the lymph nodes of the body (lymphadenopathy), most often those of the neck (cervical lymphadenopathy). In some cases, abnormal accumulation of histiocytes may occur in other areas of the body besides the lymph nodes (extranodal). These areas include the skin, central nervous system, kidney, and digestive tract. The symptoms and physical findings associated with Rosai-Dorfman disease vary depending upon the specific areas of the body that are affected. The disorder predominantly affects children, adolescents or young adults. The exact cause of Rosai-Dorfman disease is unknown.

Rosai-Dorfman Disease Resources

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

NORD Member Organizations:

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Other Organizations:

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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