Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, 77030, USA.
Translational Molecular Pathology Department, UT MD Anderson Cancer Center, Houston, TX, 77030, USA.
Virchows Arch. 2020 Sep;477(3):437-444. doi: 10.1007/s00428-020-02788-7. Epub 2020 Mar 9.
Hodgkin lymphoma is a B cell neoplasm characterized by Hodgkin and Reed-Sternberg (HRS) cells in an inflammatory background. Classic Hodgkin lymphoma (CHL) accounts for approximately 90% of all cases of HL and four types are recognized in the World Health Organization (WHO) classification: lymphocyte-rich, nodular sclerosis, mixed cellularity, and lymphocyte depleted. Castleman disease (CD) is a designation used for a heterogeneous group of diseases that involve lymph nodes. Histologically, there are hyaline vascular and plasma cell variants, the latter including human herpes virus 8 (HHV8)-positive and HHV8-negative subsets. In this study, we describe three men, 45-57 years of age, one HIV-positive, who had coexistent CHL and CD. All patients had the interfollicular variant of CHL and HHV8-negative plasma cell variant CD. Immunohistochemical analysis supported the diagnosis of CHL; the HRS cells were positive for CD15, CD30, and PAX-5 (dim). In two cases, the HRS cells and the plasma cells of CD expressed interleukin-6 (IL-6). Our review of the literature identified 34 cases of coexistent CHL and CD reported previously. In aggregate, about two-thirds of all cases of CHL have been the interfollicular variant and around 90% of CD cases were plasma cell variant, HHV8-negative in the subset of cases tested. We suggest that interfollicular variant CHL and plasma cell variant CD may be a distinct entity with a common pathogenesis, possibly related to IL-6 dysregulation. The few cases in the literature describing other forms of CHL and hyaline vascular variant CD are different from the entity reported here, with a different pathogenesis, likely similar to focal Castleman-like changes that have been described in association with various types of non-Hodgkin lymphoma.
霍奇金淋巴瘤是一种 B 细胞肿瘤,其特征为炎症背景下的霍奇金和里德-斯特恩伯格(HRS)细胞。经典型霍奇金淋巴瘤(CHL)约占所有 HL 病例的 90%,在世界卫生组织(WHO)分类中可识别出四种类型:富含淋巴细胞型、结节性硬化型、混合细胞型和淋巴细胞减少型。血管滤泡性淋巴结增生症(CD)是用于一组涉及淋巴结的异质性疾病的命名。组织学上,存在透明血管型和浆细胞型变体,后者包括人类疱疹病毒 8(HHV8)阳性和 HHV8 阴性亚群。在这项研究中,我们描述了三名年龄在 45-57 岁之间的男性,其中一名 HIV 阳性,他们同时患有 CHL 和 CD。所有患者均存在滤泡间变异型 CHL 和 HHV8 阴性浆细胞变异型 CD。免疫组织化学分析支持 CHL 的诊断;HRS 细胞对 CD15、CD30 和 PAX-5(弱)呈阳性。在两种情况下,HRS 细胞和 CD 的浆细胞均表达白细胞介素 6(IL-6)。我们对文献的回顾确定了之前报道的 34 例同时存在的 CHL 和 CD。总的来说,大约三分之二的 CHL 病例为滤泡间变异型,大约 90%的 CD 病例为浆细胞变异型,在已检测的病例亚群中 HHV8 阴性。我们认为滤泡间变异型 CHL 和浆细胞变异型 CD 可能是一种具有共同发病机制的独特实体,可能与 IL-6 失调有关。文献中描述的其他形式的 CHL 和透明血管型 CD 的少数病例与这里报道的实体不同,具有不同的发病机制,可能与各种类型的非霍奇金淋巴瘤相关的局灶性 Castleman 样变化相似。