Rosai-Dorfman Disease


Diagnosis and Treatment

The diagnosis of Rosai-Dorfman (RD) is made following a biopsy of the affected tissue. A small piece of the tissue is obtained so that it can be viewed under a microscope by a pathologist. If the cells in the tissue have certain specific characteristics, the diagnosis of RD can be made. This procedure can be performed of the lymph nodes, skin, bone, liver, lung, or bone marrow. RD cells stain with S100 and CD68.

Once the diagnosis is made, the extent of disease should be established. Further diagnostic testing will depend on the individual case and may include testing similar to that used for Langerhans cell histiocytosis: Physical exam, blood and urine tests, and x-ray procedures such as CT, MRI, ultrasound, and/or bone scanning. The severity of the disease is determined according to the number of lymph node groups affected and the sites outside of the lymph nodes.

On occasion, the immunoglobulin (antibody levels) in the blood is very high and they may need to be removed from the blood by a treatment called plasmapheresis. These levels should be checked as part of the diagnostic investigation.

Treatment depends upon the individual patient and is planned after thorough testing to determine the extent of disease. Ideal treatment, however, has not been established, and there is no ongoing clinical trial. 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. Rarely radiation therapy may be used. Chemotherapy may include vinblastine, 6-MP, methotrexate, thalidomide, or Gleevec. The ultimate goal of an overall treatment plan, of course, is to use as little treatment as possible to keep the disease under control and preserve quality of life.

Rosai-Dorfman does not usually threaten life or organ function. It is believed that 5% to 10% of patients have progressive disease that may damage tissue. However, for most patients, the disease is self-limited, and the outcome is good.

 

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