Research progress into Castleman disease / 中国肿瘤临床

ABSTRACT

Castleman disease (CD) is a rare polyclonal lymphoproliferative disorder characterized by enlarged hyperplastic lymph node(s). Unicentric Castleman disease (UCD) is an indolent condition often treated by local approaches. On the contrary, pa-tients with multicentric Castleman disease (MCD) have less favorable prognoses and require systemic treatments. Cytotoxic chemother-apy has been widely used to treat MCD with varying degrees of response because of the toxicity risk of the treatment. The pathogenesis remains unknown;however, most pieces of evidence to date point toward human herpes virus 8 (HHV-8) and deregulated overproduc-tion of interleukin (IL-6). Discovery of putative etiologic factors and targets in MCD, particularly HHV-8, CD20, and IL-6, has translat-ed to the use of rituximab, anti-IL-6-based, and antiviral therapy. Good results have been realized through targeting HHV-8 replication, CD20, and IL-6 pathways. In this article, we reviewed the classification, pathogenesis, and current treatments of CD and provided in-sights into future treatment strategies based on disease biology.