González García A, Moreno Cobo M Á, Patier de la Peña J L
Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal, Madrid, España.
Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal, Madrid, España.
Rev Clin Esp (Barc). 2016 Apr;216(3):146-56. doi: 10.1016/j.rce.2015.11.008. Epub 2015 Dec 31.
Castleman's disease is not just a single disease but rather an uncommon, heterogeneous group of nonclonal lymphoproliferative disorders, which have a broad spectrum of clinical expression. Three histological types have been reported, along with several clinical forms according to clinical presentation, histological substrate and associated diseases. Interleukin-6, its receptor polymorphisms, the human immunodeficiency virus and the human herpes virus 8 are involved in the etiopathogenesis of Castleman's disease. The study of this disease has shed light on a syndrome whose incidence is unknown. Despite recent significant advances in our understanding of this disease and the increasing therapeutic experience with rituximab, tocilizumab and siltuximab, there are still difficult questions concerning its aetiology, prognosis and optimal treatment.
卡斯特曼病并非单一疾病，而是一组罕见的、异质性的非克隆性淋巴增殖性疾病，临床表现广泛。根据临床表现、组织学基础和相关疾病，已报道了三种组织学类型以及几种临床形式。白细胞介素-6、其受体多态性、人类免疫缺陷病毒和人类疱疹病毒8参与了卡斯特曼病的发病机制。对这种疾病的研究揭示了一种发病率未知的综合征。尽管最近我们对这种疾病的认识有了显著进展，并且使用利妥昔单抗、托珠单抗和西妥昔单抗的治疗经验不断增加，但关于其病因、预后和最佳治疗仍存在难题。
