Bertinchamp R, Terriou L
Service de médecine interne et immunologie clinique, CHU Antoine-Béclère, Clamart, France.
Service de médecine interne et immunologie clinique, Centre de référence des maladies auto-immunes systémiques rares du nord et nord-ouest de France (CeRAINO), CHU de Lille, Lille, France.
Rev Med Interne. 2022 Dec;43(10S1):10S4-10S9. doi: 10.1016/S0248-8663(23)00019-X.
Castleman disease is a group of rare disorders characterized by lymph node enlargement, specific microscopic changes to the lymph nodes, and a broad range of symptoms and laboratory findings. The two main subtypes are unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). The multicentric subtype can be further classified into two categories: HHV-8 positive multicentric Castleman disease and idiopathic multicentric Castleman disease (iMCD). In the United States (US), the annual incidence of Castleman disease (CD) has been estimated to range from 6500 to 7700 in a 2014 study. Approximately 75 percent were estimated to be unicentric CD and the remaining 25 percent were estimated to be split between HHV-8-associated MCD or HHV-8-negative/idiopathic MCD. Diagnostic criteria for iMCD have been established by an international working group of pediatric and adult pathology and clinical experts. The proposed consensus criteria require characteristic histopathologic findings on lymph node biopsy, enlargement of multiple lymph node regions, the presence of multiple clinical and laboratory abnormalities, and the exclusion of infectious, malignant, and autoimmune disorders that can mimic iMCD. © 2022 Published by Elsevier Masson SAS on behalf of Société nationale française de médecine interne (SNFMI).
卡斯特尔曼病是一组罕见疾病，其特征为淋巴结肿大、淋巴结特定的微观变化以及广泛的症状和实验室检查结果。两个主要亚型是单中心卡斯特尔曼病（UCD）和多中心卡斯特尔曼病（MCD）。多中心亚型可进一步分为两类：HHV - 8阳性多中心卡斯特尔曼病和特发性多中心卡斯特尔曼病（iMCD）。在美国，2014年的一项研究估计卡斯特尔曼病（CD）的年发病率在6500至7700例之间。据估计，约75%为单中心CD，其余25%估计在HHV - 8相关MCD或HHV - 8阴性/特发性MCD之间分布。iMCD的诊断标准已由儿科和成人病理学及临床专家国际工作组制定。拟议的共识标准要求淋巴结活检有特征性组织病理学发现、多个淋巴结区域肿大、存在多种临床和实验室异常，以及排除可模仿iMCD的感染性、恶性和自身免疫性疾病。© 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。
