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 cell called histiocytes. These histiocytes typically gather in the lymph nodes, but can also affect other organs and tissues throughout the body.
Synonyms for Rosai-Dorfman disease:
Rosai-Dorfman disease primarily affects children and young adults, although it can occur at any age. The exact cause of the disease is unknown, but it is believed to involve an abnormal immune response. Common symptoms include painless swelling of lymph nodes, fever, night sweats, fatigue, and weight loss.
Diagnosis of Rosai-Dorfman disease is typically made through a combination of medical history evaluation, physical examination, imaging tests (such as CT scans or MRIs), and a biopsy of the affected tissue. The histiocytes in Rosai-Dorfman disease have distinct characteristics that can be observed under a microscope.
Treatment for Rosai-Dorfman disease depends on the severity of symptoms and the organs involved. In many cases, the disease may resolve on its own without treatment. However, if treatment is necessary, options may include corticosteroids, nonsteroidal anti-inflammatory drugs (NSAIDs), chemotherapy, or radiation therapy. Surgery may be performed to remove large masses or lesions that are causing significant symptoms or complications.
While Rosai-Dorfman disease is a chronic condition, it is generally considered benign and has a good prognosis. However, in rare cases, it can lead to complications or affect vital organs, requiring ongoing monitoring and management.