Rosai-Dorfman disease (RD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare histiocytic disorder which involves the over-production of a type of white blood cell called non-Langerhans sinus histiocyte. These cells accumulate and can lead to organ damage. This occurs most-often in the lymph nodes, but may occur in other areas of the body as well. The reason that these cells over-produce is not known, although many possibilities have been considered, including viral, bacterial, infection, environmental, and genetic causes.
In 1969, two pathologists, Juan Rosai and Ronald Dorfman, reported a distinct histiocytic disorder in several patients with massive enlargement of the lymph nodes, as well as other symptoms. They named this condition sinus histiocytosis with massive lymphadenopathy and the name has since come to be known as Rosai-Dorfman disease.
The true number of RD cases is not known, although it does occur worldwide and seems to affect equal numbers of males and females. It is most commonly seen in the first 10 years of life, but it also occurs in adult patients.
Because this disease is so rare, no large research studies have been performed, and there is no established, widely accepted treatment. However, RD is usually not life-threatening, and many patients do not require treatment.
It is believed that 70% to 80% of patients have spontaneous improvement of symptoms without treatment, although they may have alternating episodes of worsening and relieving of symptoms for a long period of time. Some patients with severe or persistent disease or cases where organ function is threatened (such as breathing obstruction or kidney failure) may require treatment with surgery, steroids, and/or chemotherapy. In rare cases, radiation therapy may be used.
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