Rosai-Dorfman Disease: Signs and Symptoms

Rosai-Dorfman Disease is a rare disorder that is characterized by an overproduction and accumulation of histiocyte, a type of white blood cell, in the lymph nodes. This creates an enlargement of the lymph nodes that are benign and painless. The disease is systemic, meaning that it can affect the entire body. It occurs more commonly in the cervical area, but it can occur in other areas of the body besides the lymph nodes such as the central nervous system, eyes, upper respiratory tract, skin and kidney. Rosai-Dorfman was first described in 1969 by Rosai and Dorfman. The disease is also known as Sinus Histiocytosis with Massive Lymphadenopathy and Destombes-Rosai-Dorfman disease.

SIGNS AND SYMPTOMS

The signs and symptoms of Rosai-Dorfman disease include enlarged lymph nodes (Lymphadenopathy), enlarged liver, enlarged spleen, fever, pallor, anemia, Rheumatic symptoms, weight loss, tonsillitis, nasal discharge, nasal obstruction and white blood cell accumulation in the skin, lungs, bones, eye orbits, eyelids, kidneys, peritoneum, testes, central nervous system or digestive tract.

CAUSES

The cause of Rosai-Dorfman disease is unknown, but there is a possibility that it is caused by an infectious agent or an autoimmune disease. Although it remains uncertain if the disease is caused by an infectious agent or autoimmune disease, it is still classified as a reactive disorder.

DIAGNOSIS, TREATMENT AND PROGNOSIS

Rosai-Dorfman disease is usually diagnosed by doing a lymph node biopsy. Other tests that aid in the diagnosis include CT scans, ultrasounds and MRI scans. Most patients with a mild case of the disease have a spontaneous remission after several years, usually with no treatment. Treatment for more severe cases includes surgery, radiotherapy or medications such as corticosteroids or cyclophosphamide. Even if the treatment is successful there is still a possibility for the disease to reoccur. Prognosis is usually good, but severe cases can result in death especially if multiple organs are involved or there is an underlying autoimmune dysfunction present.

DISEASE ASSOCIATION