Definition of Castleman disease

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Castleman disease: A disorder of lymphoid tissue (lymphadenopathy) with massive overgrowth (hyperplasia) of lymph nodes ("swollen glands"), most commonly affecting the nodes between the lungs (in the mediastinum).

There are two quite different forms of Castleman's disease. One form, found in 90% cases, is a localized kind of lymphadenopathy. It usually causes no symptoms, follows an indolent (lazy) course and can be cured simply by excision (surgical removal).

The far more ominous form of Castleman's disease, encountered in 10% of cases, is a multicentric lymphadenopathy with progressive systemic signs and symptoms such as fever and anemia. This rapidly progressive multicentric type of Castleman's disease is associated with infection by human herpesvirus 8 (HHV-8).

The disease was first described by B. Castleman's and colleagues in 1956.