[Castleman's disease, a fatal cause of lymph node enlargement and fever]. / De ziekte van Castleman: een fatale oorzaak van lymfkliervergroting en koorts.

A 71-year old Turkish man with fever, night sweats and generalized lymphadenopathy was diagnosed as having multicentric Castleman's disease. This is a rare and often fatal cause of lymph node enlargement and fever. Histological investigation confirms the diagnosis, but the morphological features closely resemble reactive lymphadenopathy or lymphoma. Infection with human herpes virus 8 is associated with Castleman's disease, in both hiv-positive and hiv-negative patients. An interleukin-6 mediated immune response against HHV-8 seems to play an important role in the pathogenesis of the multicentric form of Castleman's disease. The disease is usually rapidly progressive, but can have a milder course. There is no standard treatment: usually systemic chemotherapy in combination with steroids is applied..Recently, promising results have been obtained using rituximab and anti-IL-6-receptor antibodies.