Castleman病的临床病理特征及可能的发病机制

Clinicopathological features and possible pathogenesis of Castleman disease

Castleman病(Castleman disease,CD)是一类高度临床异质性和特征病理反应性淋巴结增生性疾病,较为少见,目前病因及发病机制尚未明确。单中心型(unicentric CD,UCD)的致病机制很可能是由肿瘤间质细胞克隆性增殖及获得性突变引起的。特发性多中心型(idiopathic multicentric CD,iMCD)在临床表现和病理特征上与多种疾病相交叉,已发现的较常见的致病因素有自身免疫性因素、副肿瘤综合征、病毒感染性因素以及炎症因子的失调。iMCD的致病机制很可能由多种病因综合作用引起免疫调节失调和细胞因子增加的共同途径产生。本文总结近年来CD最新病因及发病机制,旨在加深对该疾病的认识。

Castleman disease(CD) refers to a group of uncommon lymph node proliferative disorders that have highly clinically heterogeneous and obvious pathological features. The etiology and pathogenesis of CD remain unclear. The pathogenesis of unicentric CD(UCD) is most likely driven by clonal proliferation and acquired mutations of tumor stromal cells. Idiopathic multicentric CD(iMCD) intersects with a variety of diseases in terms of its clinical manifestation and pathological features including autoimmune factors, paraneoplastic syndrome, viral infectious factors and inflammatory factor disorders. The pathogenesis of iMCD is probably the common pathway of immune dysregulation and cytokine increase caused by the combined action of multiple etiologies. Here, we aim to summarize the latest reported etiology and pathogenesis of CD, aiming to deepen the understanding of this disease.