Kramer Steven, Uppal Guldeep, Wang Zi-Xuan, Gong Jerald Z
Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, PA.
Appl Immunohistochem Mol Morphol. 2019 Jan;27(1):8-14. doi: 10.1097/PAI.0000000000000527.
Non-Hodgkin lymphoma may occasionally contain large transformed cells resembling Hodgkin and Reed-Sternberg cells (HRS cells). We report a 63-year-old man with HRS cells in a recurrent mantle cell lymphoma (MCL). The patient initially presented with orbital MCL and recurred after 8 years with widespread involvement. The HRS cells were present in the recurrent disease but not in the initial orbital lesions, suggesting a transformed event after a prolonged disease course. Morphologically, the HRS cells were single cells and small clusters among the MCL cells and were frequently accompanied by histiocytes but without eosinophils or other inflammatory cells. The HRS cells showed a phenotype of classic Hodgkin lymphoma (cHL). The HRS cells were clonally related to the MCL, which was demonstrated by the presence of identical t(11;14) that resulted in productive cyclin D1 expression in both cell types. Review of the literature identified 7 additional MCL cases that showed a spectrum of clinical and pathologic features ranging from scattered HRS cells to true composite MCL and cHL. The HRS cells were clonally related to MCL in 4 cases (including the current case) and unrelated in 2 cases. These findings suggest MCL with HRS cells is a heterogeneous group that may represent a spectrum of transformation at the various stages. Proof of clonal relationship between HRS cells and MCL is useful to distinguish these cases from true composite MCL and cHL.
非霍奇金淋巴瘤偶尔可能含有类似于霍奇金和里德-斯腾伯格细胞(HRS细胞)的大转化细胞。我们报告了一名63岁患有复发性套细胞淋巴瘤(MCL)伴HRS细胞的男性患者。该患者最初表现为眼眶MCL,8年后复发且病变广泛。HRS细胞出现在复发病变中,但最初的眼眶病变中未出现,提示在疾病长期发展后发生了转化事件。形态学上,HRS细胞为单个细胞及小细胞簇,位于MCL细胞之间,常伴有组织细胞,但无嗜酸性粒细胞或其他炎症细胞。HRS细胞表现出经典霍奇金淋巴瘤(cHL)的表型。HRS细胞与MCL存在克隆相关性,这通过两种细胞类型中均存在导致细胞周期蛋白D1有效表达的相同t(11;14)得以证明。文献回顾发现另外7例MCL病例,其临床和病理特征范围从散在的HRS细胞到真正的复合性MCL和cHL。4例(包括本病例)中的HRS细胞与MCL存在克隆相关性,2例无相关性。这些发现表明伴HRS细胞的MCL是一个异质性群体,可能代表了不同阶段的一系列转化。证明HRS细胞与MCL之间的克隆关系有助于将这些病例与真正的复合性MCL和cHL区分开来。