Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.
Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.
J Clin Exp Hematop. 2022 Jun 28;62(2):60-72. doi: 10.3960/jslrt.21036. Epub 2022 Apr 27.
Castleman disease consists of several lymphoproliferative subtypes that share some histological features in the lymph nodes. On the other hand, numerous clinical findings and etiologies make the disease challenging to understand. The origin of the disease is the hyaline vascular-type unicentric Castleman disease (UCD), first reported by Benjamin Castleman et al. in 1954. Although UCD is characterized by localized lesions and lack of symptoms, multicentric Castleman disease (MCD) with multiple lesions and systemic symptoms was reported by Frizzera in 1983. MCD is further divided according to KSHV/HHV8 infection status. In KSHV/HHV8-related MCD, viral infection signals lead to excessive cytokine production, and cause clinical and pathologic abnormalities. Some cases of plasma cell-type KSHV/HHV8-negative MCD can be found in association with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes), which is a paraneoplastic syndrome. The others are idiopathic MCD, which are currently considered a heterogeneous group of diseases with overlapping pathological and clinical features. In this article, we summarize the historical evolution of Castleman disease to help understand the disease concept. We also review the latest ideas and definitions of the subtypes within the MCD spectrum and summarize the histopathological findings.
血管滤泡性淋巴结组织增生症（Castleman disease）由几种淋巴组织增生性亚型组成，这些亚型在淋巴结中具有一些组织学特征。另一方面，许多临床表现和病因使得该病难以理解。该病的起源是透明血管型局灶性 Castleman 病（unicentric Castleman disease，UCD），由 Benjamin Castleman 等人于 1954 年首次报道。虽然 UCD 以局部病变和缺乏症状为特征，但多中心 Castleman 病（multicentric Castleman disease，MCD）伴有多部位病变和全身症状，于 1983 年由 Frizzera 报道。MCD 根据是否存在 KSHV/HHV8 感染进一步分类。在 KSHV/HHV8 相关的 MCD 中，病毒感染信号导致过度细胞因子产生，引起临床和病理异常。一些浆细胞型 KSHV/HHV8 阴性 MCD 病例可与 POEMS 综合征（多发性神经病、器官肿大、内分泌病、M 蛋白和皮肤改变）相关，这是一种副肿瘤综合征。其他为特发性 MCD，目前被认为是一组具有重叠病理和临床特征的异质性疾病。本文总结了 Castleman 病的历史演变，以帮助理解该病概念。我们还回顾了 MCD 谱内各亚型的最新观点和定义，并总结了组织病理学发现。
