Paulli Marco, Neri Giuseppe, Antoci Francesca, D'Este Edoardo, Minetto Marco, Carpi Federico, Fauci Martina La, Gambacorta Marcello, Lucioni Marco, Arcaini Luca
Department of Molecular Medicine, University of Pavia, Pavia, Italy.
Division of Anatomic Pathology Unit, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Ann Hematol. 2025 Sep 6. doi: 10.1007/s00277-025-06527-3.
Castleman disease (CD) is a rare lymphoproliferative disorder with unique clinicopathological features, including two distinct clinical subtypes categorized as unicentric (UCD) and multicentric (MCD). UCD usually involves a single lymph node site presenting with no or minimal local symptoms. Histologically, most UCD cases exhibit regressive hyaline vascular germinal centers, characterized by penetrating vessels, dendritic hyperplasia/dysplasia, and increased interfollicular vascularity. While complete surgical excision is the standard treatment for UCD, cases treated with neoadjuvant anti-CD20 therapy have been reported. Here, we describe a UCD case treated with anti-CD20, that showed only a partial response. We detail the clinical findings and the histological changes observed in the surgical specimen following therapy, and discuss the potential influence of the local microenvironment on the therapeutic efficacy of anti-CD20.
卡斯特曼病(CD)是一种罕见的淋巴增生性疾病,具有独特的临床病理特征,包括分为单中心型(UCD)和多中心型(MCD)的两种不同临床亚型。UCD通常累及单个淋巴结部位,表现为无局部症状或局部症状轻微。组织学上,大多数UCD病例表现为退行性透明血管生发中心,其特征为穿透性血管、树突状细胞增生/发育异常以及滤泡间血管增多。虽然完整手术切除是UCD的标准治疗方法,但也有报道采用新辅助抗CD20治疗的病例。在此,我们描述一例接受抗CD20治疗的UCD病例,该病例仅表现出部分缓解。我们详细阐述了治疗后手术标本中的临床发现和组织学变化,并讨论了局部微环境对抗CD20治疗效果的潜在影响。