Rosai-Dorfman Disease: Signs and Symptoms
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
Rosai-Dorfman Disease is associated with Amyloidosis, Anemia, Autoimmune lymphoproliferative syndrome, HIV, Lymphoma, Homer's Syndrome, Human Herpes Virus and Macrophages. Rosai-Dorfman is often confused with Lymphoma, a cancer that affects part of the immune system known as the lymphatic system.
PREVALENCE
Rosai-Dorfman Disease is more common in people of African or Caribbean descent, adolescents and young adults. The typical age of onset is usually around the age of 20, but it can occur at any age and males are affected slightly more than females.
Published by Cristina Olvera
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- The cause of Rosai-Dorfman disease is unknown.
- Rosai-Dorfman disease is usually diagnosed by doing a lymph node biopsy.
- Prognosis is usually good, but severe cases can result in death.