Guastafierro Vincenzo, Marchiori Deborah, Bonometti Arturo, Rahal Daoud, Fraticelli Sara, Grion Giulia, Campiotti Leonardo, Uccella Silvia
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, Milan, 20072, Italy.
Department of Pathology, IRCCS Humanitas Research Hospital, Via Manzoni 56, Rozzano, Milan, 20089, Italy.
Virchows Arch. 2025 Sep 12. doi: 10.1007/s00428-025-04251-x.
Castleman disease (CD) is a rare, heterogeneous, lymphoproliferative disorder that typically involves lymph nodes or, less commonly, extranodal sites such as the spleen. Based on clinical presentation CD is categorized into unicentric (UCD) and multicentric (MCD) forms, the latter further classified into HHV8-related, POEMS-associated, and idiopathic forms. We report two cases of HHV8-related MCD diagnosed on splenectomy specimens from patients presenting with splenomegaly, lymphadenopathies, and B symptoms. Histopathological analysis revealed mixed hyaline-vascular and plasma cell patterns with HHV8-positive lymphoid cells, and, in one case, an associated Kaposi sarcoma in splenic hilum lymph nodes. A systematic review of the literature identified 27 additional cases of CD diagnosed on splenectomy, which were analyzed alongside our two cases. Our study highlights that histologic evaluation of spleen tissue reliably reflects nodal CD patterns in both unicentric and multicentric forms and that, in selected cases, splenectomy remains a valuable diagnostic tool in Castleman disease.
卡斯特曼病（CD）是一种罕见的、异质性的淋巴增生性疾病，通常累及淋巴结，较少累及脾等结外部位。根据临床表现，CD分为单中心型（UCD）和多中心型（MCD），后者进一步分为与HHV8相关型、与POEMS综合征相关型和特发型。我们报告了2例经脾切除术标本诊断为与HHV8相关的MCD病例，患者表现为脾肿大、淋巴结病和B症状。组织病理学分析显示，存在混合的透明血管和浆细胞模式，伴有HHV8阳性淋巴细胞，其中1例在脾门淋巴结伴有卡波西肉瘤。对文献进行系统回顾后，又确定了27例经脾切除术诊断的CD病例，并与我们的2例病例一起进行了分析。我们的研究强调，脾组织的组织学评估能够可靠地反映单中心型和多中心型CD的淋巴结模式，并且在某些特定病例中，脾切除术仍是卡斯特曼病的一种有价值的诊断工具。
