Sevilla-Lizcano Diana Brisa, Frias-Soria Christian Lizette, Ortiz-Hidalgo Carlos
Departamento de Patología Quirúrgica y Molecular, Centro Médico ABC.
Departamento de Biología Celular y Tisular, Universidad Panamericana, Ciudad de México, México.
Gac Med Mex. 2017;153(5):550-558. doi: 10.24875/GMM.17003021.
Castleman disease (CD) is a rare lymphoproliferative that comprises two distinct clinical subtypes (unicentric and multicentric) and has two basic histopathology patterns that are hyaline-vascular (HV) and plasma-cell (PC) type. Some cases of multicentric PC disease are associated with HHV-8 infection.
To present the histopathologic and immunohistochemical characteristics of 39 cases of CD.
A review of cases with the diagnosis CD from the files of the Department of Pathology of the ABC Medical Centre in Mexico City was performed. Thirty-nine cases of CD were identified, and a detailed paraffin immunophenotypic study of 9 of them was completed using desmin, cytokeratin OSCAR (CO) and Epidermal growth factor receptor (EGFR), to evaluate the dendritic cell population.
Of the 39 cases of CD, 24 were HV and 15 CP. All HV cases were unicentric and only one case of CP was multicentric. The most frequent localization in both subtypes was in lymph nodes; 21/24 cases in HV and 15 cases of CP. All cases were immunostained with CD20 that was expressed in the germinal centers (CGs), CD3 in the paracortical zone, and CD21 in follicular dendritic cells (CDF) within CGs, with expansion towards the area of the hyperplastic mantle zone (only in the HV variant). One case of CD CP was positive for HHV-8. Of the nine cases (6 HV and 3 PC cases) that were detailed with IHC, we found EGFR expression in FDC in all but one of the 9 cases studied and desmin was positive in fibroblastic reticulum cells (FRC) in all, but one of the cases of CD. CO was positive FRC in 3 of 6 cases of HV type and all (3) of the PC type. Clinical, histopathological and HIV and HHV-8 status markers, allow for the classification of CD into groups with markedly different outcomes and disease associations.
卡斯特曼病（CD）是一种罕见的淋巴增殖性疾病，包括两种不同的临床亚型（单中心型和多中心型），具有两种基本的组织病理学模式，即透明血管型（HV）和浆细胞型（PC）。一些多中心PC病病例与HHV - 8感染有关。
介绍39例CD的组织病理学和免疫组化特征。
对墨西哥城ABC医疗中心病理科档案中诊断为CD的病例进行回顾。共鉴定出39例CD，并对其中9例进行了详细的石蜡免疫表型研究，使用结蛋白、细胞角蛋白OSCAR（CO）和表皮生长因子受体（EGFR）来评估树突状细胞群体。
39例CD中，24例为HV型，15例为CP型。所有HV型病例均为单中心型，仅1例CP型为多中心型。两种亚型最常见的定位均在淋巴结；HV型21/24例，CP型15例。所有病例均用CD20免疫染色，其在生发中心（CGs）表达，CD3在副皮质区表达，CD21在CGs内的滤泡树突状细胞（CDF）表达，并向增生性套区扩展（仅在HV型变体中）。1例CD CP型病例HHV - 8呈阳性。在通过免疫组化详细研究的9例病例（6例HV型和3例PC型）中，我们发现除1例研究病例外，其余所有病例的FDC中均有EGFR表达，并且除1例CD病例外，其余所有病例的成纤维网状细胞（FRC）中结蛋白均呈阳性。CO在6例HV型病例中的3例以及所有（3例）PC型病例的FRC中呈阳性。临床、组织病理学以及HIV和HHV - 8状态标志物有助于将CD分为结局和疾病关联明显不同的组。
