Meignin V, Parrens M
Département de pathologie, Hôpital Saint-Louis, université de Paris, Paris, France.
Service de pathologie, hôpital Haut-Lévêque, CHU de Bordeaux, université de Bordeaux, Bordeaux, France.
Rev Med Interne. 2022 Dec;43(10S1):10S10-10S16. doi: 10.1016/S0248-8663(23)00020-6.
Histologically, Castleman's disease associates three subtypes: 1-the vascular hyaline (HV) subtype more often seen in unicentric forms; 2-the plasmacytic (PV) subtype, more frequently associated with the HHV8+ and idiopathic multicentric form; 3-the mixed subtype associating both HV and PV aspects that may be encountered in any type of Castleman's disease. If the diagnosis of unicentric (isolated mass) and multicentric HHV8+ Castleman's disease is easy, the diagnosis of the idiopathic multicentric form remains particularly difficult because it is at the crossroads of many other pathologies (infectious, tumoral and dysimmune), making an anatomoclinical comparison necessary. The role of the pathologist, in the context of disseminated lesions (polyadenopathy and splenomegaly), is to identify lesions that may be part of Castleman's disease, to systematically perform HHV8 testing and to perform complete phenotyping associated with molecular analysis (B and T-cell clonality) in order to rule out a lymphomatous process and certain infectious etilogies. In all cases, its role will be a warning bell and the diagnosis of Castleman's disease will be retained only after a rigorous anatomic and clinical confrontation. © 2022 Published by Elsevier Masson SAS on behalf of Société nationale française de médecine interne (SNFMI).
从组织学上看，卡斯特曼病有三种亚型：1. 血管透明变性（HV）亚型，更常见于单中心形式；2. 浆细胞（PV）亚型，更常与HHV8阳性和特发性多中心形式相关；3. 混合亚型，兼具HV和PV特征，可出现在任何类型的卡斯特曼病中。如果单中心（孤立肿块）和多中心HHV8阳性卡斯特曼病的诊断容易，那么特发性多中心形式的诊断仍然特别困难，因为它处于许多其他病理情况（感染性、肿瘤性和免疫失调性）的交叉点，因此进行解剖临床比较是必要的。在出现播散性病变（多腺病和脾肿大）的情况下，病理学家的作用是识别可能属于卡斯特曼病的病变，系统地进行HHV8检测，并进行与分子分析（B细胞和T细胞克隆性）相关的完整表型分析，以排除淋巴瘤过程和某些感染病因。在所有情况下，其作用将是一个警钟，只有在经过严格的解剖和临床对照后，卡斯特曼病的诊断才能成立。© 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。
