摘要
Castleman病(CD)是一种罕见的淋巴增生性疾病,临床上分为单中心型(UCD)和多中心型(MCD)。UCD病情较轻,治疗主要依靠手术,预后较好;MCD多有全身症状,迄今尚缺乏标准的治疗方案,5年死亡率高达35%。过去,MCD的治疗方法包括等待观察、糖皮质激素治疗、化疗(联合或不联合利妥昔单抗)、免疫调节治疗等。近年来,随着针对MCD病因研究的进展,白细胞介素-6(IL-6)和人类疱疹病毒-8(HHV-8)在MCD发病中的重要作用得到了进一步明确,也涌现出一些针对上述靶点(尤其是IL-6)的新型治疗手段。本文回顾了MCD的治疗现状和最新进展,希望能提高临床医师对于此种罕见病的认识和诊疗水平。
Castleman′s disease（CD）is a rare lymphoproliferative disorder which comprises two distinct clinical subtypes：unicentric CD（UCD）and multicentric CD（MCD）. Unlike UCD,which is a curable disease with surgical resection,MCD is a disease with systemic symptoms and high mortality rate（the 5-year mortality rate could be as high as 35%）. There is no standard of care for MCD. Current treatment options include observation,glucocorticoids,chemotherapy（with or without rituximab）and immunomodulatory agents. Progress have been made during the recent years,interleukin-6（IL-6）and human herpes virus 8（HHV-8）have been recognized as key factors involved in the pathogenesis of MCD. Accordingly,drugs targeting these factors（especially IL-6）have been developed to treat MCD. In this paper,we try to review the current treatment options and new emerging therapies for MCD which might help Chinese clinicians to learn more about this rare disease.
出处
《国际药学研究杂志》
CAS
CSCD
北大核心
2017年第2期162-166,共5页
Journal of International Pharmaceutical Research
基金
国家重点研发计划精准医学研究重点专项"罕见病临床队列研究"资助项目(2016YFC0901500)
北京协和医院杰出青年基金项目(JQ201508)
