Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare non-cancerous disorder characterized by the overproduction and accumulation of a specific type of immune cells called histiocytes. These histiocytes typically gather in the lymph nodes, although they can also affect other organs and tissues throughout the body.
The exact cause of Rosai-Dorfman disease remains unknown, but it is believed to involve an abnormal immune response. Symptoms of the condition may vary depending on the organs affected, but commonly include painless swelling of lymph nodes, fever, night sweats, fatigue, and weight loss.
Diagnosis of Rosai-Dorfman disease involves a combination of medical history evaluation, physical examination, imaging tests, and biopsy of affected tissues. While there is no specific cure for the disease, treatment options aim to manage symptoms and may include corticosteroids, chemotherapy, radiation therapy, or surgical removal of affected tissues.
Rosai-Dorfman disease is generally self-limiting and has a good prognosis, with most cases resolving spontaneously or with treatment. However, in rare instances, the disease can become chronic or recur. Regular follow-up with healthcare professionals is important to monitor the condition and manage any potential complications.