Rosai-Dorfman disease
Overview
A rare condition characterized by excessive production and accumulation of a particular white blood cell (histiocyte). Accumulation primarily occurs in the lymph nodes, especially in the neck, but may also occur in the skin, central nervous system, digestive tract and kidneys.
Symptoms
* Enlarged lymph nodes * Enlarged liver * Enlarged spleen * Fever * Pallor * Anemia * Rheumatic symptoms * Weight loss * Tonsillitis * Nasal discharge * Nasal obstruction * White blood cell accumulation in skin * White blood cell accumulation in lung * White blood cell accumulation in bone * White blood cell accumulation in eye orbits * White blood cell accumulation in eyelids * White blood cell accumulation in kidneys * White blood cell accumulation in peritoneum * White blood cell accumulation in testes * White blood cell accumulation in central nervous system * White blood cell accumulation in digestive tract
Prognosis
* Often quick and complete spontaneous resolution * Sometimes protracted clinical course for years or decades: particularly if widespread extranodal involvement * Sometimes disappears and reappears years later at another site * Sometimes death due to: extensive disease affecting vital organs complications related to immunologic abnormalities,40,41 such as amyloidosis42