Definition of Castleman disease

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 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 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 disease is associated with infection by human herpesvirus 8 (HHV-8).

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

Castleman disease also is referred to as:

• Castleman's disease
• Angiofollicular lymph node hyperplasia
• Giant lymph node hyperplasia