[Advances in etiology and management of Castleman's disease]

Abstract

Castleman's disease (CD) is a rare lymphoproliferative disorder. The etiology of CD may involve viral infection, abnormal modulation of cytokines, and angiogenesis. Human herpes virus (HHV) -8 infection and interleukin-6 (IL-6) overexpression may play key roles in the development of CD. Treatment options include surgical excision, radiation therapy, chemotherapy, antiviral therapy, and targeted therapy. No standardized treatment has been established for multicentric CD and the treatment efficacy usually is poor. Among newly available agents, the effectiveness of antiviral therapy against HHV-8 is unclear; anti-CD20 and anti-IL-6 receptor monoclonal antibodies have shown promising efficacy; thalidomide and bortezomib have shown their initial efficacy.