Gasljevic Gorana, Bonometti Arturo, Anagnostopoulos Ioannis, Balaguè Olga, Van den Brand Michiel, Cook James R, Laurent Camille, Ponzoni Maurilio, Quintanilla-Martinez Leticia, Sander Birgitta, Dirnhofer Stefan
Department of Pathology, Institute of Oncology Ljubljana, Zaloška Cesta 2, 1000, Ljubljana, Slovenia.
Medical Faculty, University of Maribor, Taborska cesta 8, Maribor, Slovenia.
Virchows Arch. 2025 Jul 12. doi: 10.1007/s00428-025-04171-w.
Castleman disease (CD) is an intriguing and complicated group of local and systemic disorders mainly affecting lymph nodes with heterogeneous presentation and therapeutic needs. These disorders were the topic of Session 1 of the Lymphoma Workshop at the 2024 EA4HP in Dubrovnik, Croatia. In this report, we summarize the features of the 85 submitted cases and review the differential diagnosis, pitfalls, and advances for all CD subtypes. Specifically, the molecular landscape of unicentric CD and its relationship with follicular dendritic cell proliferations and indolent T-lymphoblastic proliferation will be discussed. The spectrum of idiopathic multicentric CD (MCD), TAFRO syndrome, as well as the clinical and histopathological peculiarities of POEMS-CD, is reviewed. Cases of Kaposi sarcoma-associated herpesvirus/human herpesvirus 8 (KSHV/HHV8) + MCD were the most complicated and well demonstrated the difficulties and overlaps in the differential diagnosis of KSHV/HHV8 + lymphoproliferative disorders. Finally, the important topic of CD mimickers will be addressed, demonstrating how the integration of clinical, laboratory, histopathological, and molecular data is mandatory to confirm a diagnosis of CD and how to distinguish it from the many neoplastic, autoimmune, and infective mimickers.
卡斯特曼病(CD)是一组复杂且引人关注的局部和全身性疾病,主要累及淋巴结,临床表现和治疗需求各异。这些疾病是2024年在克罗地亚杜布罗夫尼克举行的欧洲血液学和血液病理学协会(EA4HP)淋巴瘤研讨会第一场会议的主题。在本报告中,我们总结了85例提交病例的特征,并回顾了所有CD亚型的鉴别诊断、陷阱及进展。具体而言,将讨论单中心CD的分子格局及其与滤泡树突状细胞增殖和惰性T淋巴细胞母细胞增殖的关系。还将回顾特发性多中心CD(MCD)、TAFRO综合征以及POEMS-CD的临床和组织病理学特点。卡波西肉瘤相关疱疹病毒/人类疱疹病毒8(KSHV/HHV8)阳性的MCD病例最为复杂,很好地展示了KSHV/HHV8阳性淋巴增殖性疾病鉴别诊断中的困难和重叠之处。最后,将探讨CD模仿者这一重要话题,说明如何整合临床、实验室、组织病理学和分子数据以确诊CD,以及如何将其与众多肿瘤性、自身免疫性和感染性模仿者区分开来。