Castleman病的研究进展

Castleman病（castleman disease，CD）是一类少见的以不明原因淋巴结肿大为特征的慢性多克隆性淋巴组织增生性疾病。单中心型Castleman病（unicentric CD，UCD）临床过程呈惰性，一般通过手术切除病灶而达到长期生存。相反，多中心型Castleman病（multicentric CD，MCD）预后相对较差，需全身治疗。细胞毒性药物虽广泛用于治疗MCD，但疗效报道不一。CD至今发病机制不明，但研究较多且明确的发病机制是HHV-8感染和IL-6异常分泌。近年针对这些发病机制而派生出的抗病毒治疗和靶向治疗也取得了较好的疗效，进一步为将来针对CD病理生理特点制定个体化治疗提供了依据和方向。本文将对该病的分类、发病机制和治疗取得的进展作一综述。 

Research progress into Castleman disease

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, patients with multicentric Castleman disease (MCD) have less favorable prognoses and require systemic treatments. Cytotoxic chemotherapy 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 overproduction of interleukin (IL-6). Discovery of putative etiologic factors and targets in MCD, particularly HHV-8, CD20, and IL-6, has translated 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 insights into future treatment strategies based on disease biology.