Multicentric Castleman Disease

Abstract

Multicentric Castleman disease is a systemic disorder of lymph nodes characterized by inflammatory symptoms including fever, night sweats, cachexia, malaise, and lymphadenopathy together with laboratory abnormalities including anemia and hypoalbuminemia. Together these symptoms may be life threatening. The diagnosis is based on specific pathological findings in affected lymph nodes. In individuals infected with HIV, multicentric Castleman disease is almost exclusively caused by infection with a cancer-causing herpesvirus, Kaposi sarcoma-associated herpesvirus (KSHV, also known as human herpesvirus 8). This may be considered a separate entity from the non-KSHV-related or idiopathic form of multicentric Castleman disease and from unicentric forms of Castleman disease. While these disorders have pathological and clinical similarities, the KSHV-related form is distinguished by its pathological characteristics and unique viral etiology.