Castleman病的发病机制和治疗进展

Advances in Etiology and Management of Castleman's Disease

Castleman病(CD)是一种少见的淋巴组织增殖性疾病,病因可能与病毒感染、细胞因子调节异常和血管增生等有关。近年研究发现,人类疱疹病毒(HHV)-8感染和白细胞介素(IL)-6过度表达在CD的发病机制中发挥重要作用。治疗方法包括手术切除、放疗、化疗、抗病毒治疗和靶向治疗等。多中心型CD目前尚无标准治疗方法,总体疗效欠佳。新式治疗中抗HHV-8病毒治疗效果不明确,抗CD20单抗和抗IL-6受体单抗已有良好疗效的报道,沙利度胺和硼替佐米等也初见疗效。

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 op- tions include surgical excision, radiation therapy, chemotherapy, antiviral therapy, and targeted therapy. No standardized treatment has been established for muhicentric 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.