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一名 67 岁女性,具有介于 EBV 阳性弥漫性大 B 细胞淋巴瘤和 EBV 阳性经典霍奇金淋巴瘤之间的组织学特征和免疫表型特征的恶性淋巴瘤:与老年人中 EBV 相关的“灰色区域”淋巴瘤。

A malignant lymphoma with histological features and immunophenotypic profile intermediate between EBV-positive diffuse large B-cell lymphoma and EBV-positive classical Hodgkin lymphoma in a 67-year-old female: a "gray zone" lymphoma associated with Epstein-Barr virus in the elderly.

机构信息

Department of Pathology, Duke University Medical Center, Durham, NC 27710, United States.

出版信息

Pathol Res Pract. 2012 Jun 15;208(6):363-7. doi: 10.1016/j.prp.2012.04.003. Epub 2012 May 7.

Abstract

Epstein-Barr virus (EBV) can be associated with both classical Hodgkin lymphoma (cHL) and non-Hodgkin lymphoma of the B-cell type, particularly in immunodeficient patients or elderly individuals. While polymorphic variants of EBV-positive large B-cell lymphoma (EBV+ DLBCL) frequently resemble cHL in morphology, and thereby may cause diagnostic difficulty, a true gray zone lymphoma with overlapping morphological and immunophenotypical features of EBV+ DLBCL and EBV+ cHL has not been reported in the literature. We describe a unique case of an EBV+ malignant lymphoma of B-cell origin with hybrid features of EBV+ DLBCL and EBV+ cHL in a 67-year-old female without an identifiable etiology for immunodeficiency. The biopsy of an enlarged lymph node showed a polymorphic infiltrate containing Reed-Sternberg-like pleomorphic large cells, which were positive for CD30 and CD15. Although CD20 was negative and PAX5 and CD45 were down-regulated, the pleomorphic large cells expressed multiple other B-cell antigens which are characteristically absent in cHL. EBV-encoded RNA hybridization (EBER) studies demonstrated nuclear reactivity in the large cells as well as in the smaller bystander cells. A clonal rearrangement of the immunoglobulin heavy chain gene was also detected by PCR. Although the results of the EBV and genotypic studies suggest this case may be an example of EBV+ DLBCL of the elderly instead of EBV+ cHL, the immunophenotype is strikingly ambiguous. Thus, this case may represent an interface between EBV+ DLBCL and EBV+ cHL.

摘要

EB 病毒(EBV)可与经典霍奇金淋巴瘤(cHL)和 B 细胞型非霍奇金淋巴瘤相关,尤其在免疫缺陷患者或老年患者中。虽然 EBV 阳性大 B 细胞淋巴瘤(EBV+ DLBCL)的多形性变异在形态上常类似于 cHL,从而可能导致诊断困难,但在文献中尚未报道具有 EBV+ DLBCL 和 EBV+ cHL 重叠形态学和免疫表型特征的真正灰色区域淋巴瘤。我们描述了一例 67 岁女性的独特 EBV 阳性 B 细胞来源恶性淋巴瘤,其具有 EBV+ DLBCL 和 EBV+ cHL 的混合特征,且无法确定其免疫缺陷的病因。肿大淋巴结的活检显示,一种多形性浸润物含有 Reed-Sternberg 样多形性大细胞,这些细胞 CD30 和 CD15 阳性。尽管 CD20 阴性,PAX5 和 CD45 下调,但多形性大细胞表达多种其他 B 细胞抗原,这些抗原在 cHL 中特征性缺失。EBV 编码的 RNA 杂交(EBER)研究显示大细胞和较小旁观者细胞的核反应性。通过 PCR 还检测到免疫球蛋白重链基因的克隆重排。尽管 EBV 和基因型研究的结果表明,该病例可能是老年 EBV+ DLBCL 的一个例子,而非 EBV+ cHL,但免疫表型非常模糊。因此,该病例可能代表 EBV+ DLBCL 和 EBV+ cHL 之间的界面。

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