Rosai–Dorfman disease
Rosai–Dorfman disease (pronounced roh-zay dor-fman), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare, benign disease that is characterized by an overproduction of a type of white blood cell known as a histiocyte. The disease was first described in 1969 by the pathologists Juan Rosai and Ronald Dorfman.
Etymology
The disease is named after the two pathologists who first described it, Juan Rosai and Ronald Dorfman. The term "sinus histiocytosis with massive lymphadenopathy" describes the main features of the disease: an overproduction of histiocytes (a type of white blood cell) and enlarged lymph nodes.
Symptoms
The most common symptom of Rosai–Dorfman disease is enlarged lymph nodes, particularly in the neck. Other symptoms can include fever, weight loss, night sweats, and fatigue. In some cases, the disease can affect other parts of the body, such as the skin, eyes, and central nervous system.
Diagnosis
Diagnosis of Rosai–Dorfman disease is typically made through a tissue biopsy of an affected lymph node. The biopsy will show an overabundance of histiocytes, which is a hallmark of the disease.
Treatment
Treatment for Rosai–Dorfman disease can vary depending on the severity of the disease and the organs involved. In some cases, no treatment is necessary and the disease will resolve on its own. In other cases, treatment options can include steroids, chemotherapy, radiation therapy, or surgery to remove affected lymph nodes.
Prognosis
The prognosis for Rosai–Dorfman disease is generally good, as the disease is benign and does not typically affect a person's lifespan. However, the disease can cause significant symptoms and complications, particularly if it affects organs other than the lymph nodes.
See also
External links
- Medical encyclopedia article on Rosai–Dorfman disease
- Wikipedia's article - Rosai–Dorfman disease
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