What is Rosai-Dorfman Disease?

Rosai-Dorfman disease is a rare disorder marked by the accumulation of white blood cells, called histiocytes, that are over-produced in the body. These white blood cells typically accumulate in the person’s lymph nodes, especially those in the neck. The accumulation may also affect the other parts of the body less often. For example, this condition may affect a patient’s skin, kidneys, and gastrointestinal system; in some cases, it may affect the patient’s central nervous system as well. No one knows what causes Rosai-Dorfman disease, but it can sometimes be treated with chemotherapy, radiation, or surgery.

When a person has Rosai-Dorfman disease, histiocytes typically build up in the patient’s lymph nodes. In time, this buildup of cells often causes enlargement of the affected lymph nodes. In most cases, the enlargement is painless, and the condition is benign. It can, however, affect one part of the body or various parts of the patient’s body at once. For example, this disease may affect only the patient’s neck, or it may also affect the patient’s neck, eyes, kidneys, and even the central nervous system.

Also referred to as sinus histiocytosis with massive lymphadenopathy, this condition may not cause pain but can cause a range of other symptoms. Among the symptoms that may develop when a person has Rosai-Dorfman disease are the enlargement of the lymph nodes, liver, and spleen. Patients with this condition may also develop fever and anemia and appear pale. Some people with this condition may also experience unexplained weight loss, mucus discharge from the nose, or a stuffy nose. An individual may also develop tonsillitis or joint pain and inflammation in relation to the disease.

If a patient has a mild case of Rosai-Dorfman disease, he may not need treatment. In such a case, the condition may spontaneously resolve on its own over time. This may take years to occur, however. For severe cases, a doctor may recommend chemotherapy, radiation therapy, and sometimes even surgery.

The prognosis for a person with Rosai-Dorfman disease is generally very good. Less often, it may either cause a person's death or act as a contributing factor to it. In most cases, however, death only results from Rosai-Dorfman disease when a person is dealing with a severe case of it that affects many organs of his body. Death may also be more likely in a person who has an underlying immune system problem.