Abstract：
The spectrum of Castleman disease encompasses several different disorders. Nowadays three different forms of the disease are individualized: unicentric Castleman disease, multicentric HHV-8 associated Castleman disease and idiopathic multicentric Castleman disease. In the latter a severe form called TAFRO syndrome (thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly) tend to be individualized. Improvement in the classification and understanding of the physiopathology of CD have allowed improvement in treatment strategies. Treatment of rare but often severe manifestations, such as paraneoplastic pemphigus and bronchiolitis obliterans in unicentric CD and hemophagocytic syndrome and/or Kaposi\' sarcoma in HHV8 associated CD, are better adapted. Most of current treatment strategies are based on retrospective and very few prospective studies. Both anti-IL6/6R and anti-CD20 biotherapies have greatly improved the management of certain forms of the disease. We report in this review the most relevant studies and national or international expert consensus statements for the treatment in the different types of CD. © 2022 Published by Elsevier Masson SAS on behalf of Société nationale française de médecine interne (SNFMI).
摘要：
Castleman疾病的范围包括几种不同的疾病。如今，三种不同形式的疾病是个性化的：单中心Castleman病，多中心HHV-8相关Castleman病和特发性多中心Castleman病。在后者中，一种称为TAFRO综合征（血小板减少症，Anasarca,骨髓纤维化,肾功能不全,和器官肿大)倾向于个性化。对CD的病理生理学的分类和理解的改进允许治疗策略的改进。治疗罕见但通常严重的表现，如单中心CD中的副肿瘤性天疱疮和闭塞性细支气管炎和噬血细胞综合征和/或HHV8相关CD中的卡波西肉瘤，适应得更好。目前的治疗策略大多基于回顾性研究，很少有前瞻性研究。抗IL6/6R和抗CD20生物疗法都极大地改善了某些形式的疾病的管理。我们在这篇综述中报告了最相关的研究以及不同类型CD治疗的国家或国际专家共识声明。©2022由ElsevierMassonSAS代表法国国家石油公司（SNFMI）发布。
