Galicier L, Schleinitz N
Service d'immunopathologie clinique, hôpital Saint-Louis, 75010 Paris, France.
Service de médecine interne, hôpital La Timone, Aix-Marseille Université, 13005 Marseille, France.
Rev Med Interne. 2022 Dec;43(10S1):10S26-10S33. doi: 10.1016/S0248-8663(23)00022-X.
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).
卡斯特曼病的谱系涵盖多种不同疾病。如今，该疾病的三种不同形式已被区分开来：单中心卡斯特曼病、多中心HHV - 8相关卡斯特曼病和特发性多中心卡斯特曼病。在后者中，一种名为TAFRO综合征（血小板减少、全身性水肿、骨髓纤维化、肾功能不全和器官肿大）的严重形式往往被区分出来。卡斯特曼病分类及病理生理学认识的改善使得治疗策略得以改进。针对罕见但通常严重的表现，如单中心卡斯特曼病中的副肿瘤性天疱疮和闭塞性细支气管炎，以及HHV8相关卡斯特曼病中的噬血细胞综合征和/或卡波西肉瘤，治疗方法得到了更好的调整。当前大多数治疗策略基于回顾性研究，前瞻性研究极少。抗IL6/6R和抗CD20生物疗法都极大地改善了某些形式疾病的治疗。在本综述中，我们报告了不同类型卡斯特曼病治疗方面最相关的研究以及国家或国际专家共识声明。© 2022由爱思唯尔马松出版社代表法国国家内科医学协会（SNFMI）出版。
