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老年 EBV 阳性弥漫性大 B 细胞淋巴瘤-来自土耳其单中心的经验。

Epstein-Barr virus (EBV) positive diffuse large B cell lymphoma of the elderly-experience of a single center from Turkey.

机构信息

Department of Pathology, Ege University Faculty of Medicine, Izmir, Turkey.

出版信息

Pathol Res Pract. 2013 Aug;209(8):471-8. doi: 10.1016/j.prp.2013.04.014. Epub 2013 May 9.

DOI:10.1016/j.prp.2013.04.014
PMID:23726928
Abstract

In the 2008 WHO lymphoma classification, 'EBV-positive diffuse large B cell lymphoma (DLBCL) of the elderly is included as a new provisional entity. We aimed to evaluate the morphological, immunophenotypic, and clinical characteristics of the cases diagnosed as 'EBV-positive DLBCL of the elderly' in our center and compared them with the 'EBV-negative DLBCL' patients older than 50 years of age. EBV status was detected by Epstein-Barr early RNA (EBER) in situ hybridization analysis. By immunohistochemistry, a panel of antibodies for CD10, Bcl-2, Bcl-6, IRF4/MUM1, CD30, and Ki67 was performed. Out of 149 DLBCL patients older than 50 years, without any known history of immunodeficiency or prior lymphoma, eight patients who fulfill the criteria were re-evaluated. Five patients were male and three were female, with a median age of 67.6 years. Four patients presented with nodal involvement; others presented with bone and soft tissue, bone marrow, and spleen infiltrations. Five cases revealed predominantly monomorphic morphology, one also contained focal areas consistent with polymorphous subtype; and three patients revealed a polymorphous infiltrate. When classified according to 'Hans criteria', five were non-GCB, and three were of the GCB cell phenotype. All cases with polymorphous morphology were revealed to be of the non-GCB cell phenotype, and all expressed IRF4/MUM1. Two patients died with disease, four patients are alive and in complete remission following R-CHOP therapy, and two patients have just recently been diagnosed. When compared with the EBV-negative group, there are no reliable morphological and immunohistochemical features indicating EBV positivity. Therefore, EBER in situ hybridization analysis is necessary to identify 'EBV-positive DLBCL of the elderly'. Further studies are needed to fully understand the details of this disease, which can lead to new treatment modalities.

摘要

在 2008 年世界卫生组织淋巴瘤分类中,“老年 EBV 阳性弥漫性大 B 细胞淋巴瘤(DLBCL)”被列为一种新的暂定实体。我们旨在评估在我们中心诊断为“老年 EBV 阳性 DLBCL”的病例的形态学、免疫表型和临床特征,并将其与年龄大于 50 岁的“EBV 阴性 DLBCL”患者进行比较。EBV 状态通过 Epstein-Barr 早期 RNA(EBER)原位杂交分析进行检测。通过免疫组织化学,使用一组针对 CD10、Bcl-2、Bcl-6、IRF4/MUM1、CD30 和 Ki67 的抗体进行检测。在 149 例年龄大于 50 岁、无已知免疫缺陷或先前淋巴瘤病史的 DLBCL 患者中,重新评估了 8 例符合标准的患者。5 例为男性,3 例为女性,中位年龄为 67.6 岁。4 例患者表现为淋巴结受累;其他患者表现为骨和软组织、骨髓和脾脏浸润。5 例病例显示主要为单形性形态,1 例还包含局灶性多形性亚型区域;3 例患者显示多形性浸润。根据“Hans 标准”进行分类,5 例为非 GCB,3 例为 GCB 细胞表型。所有具有多形性形态的病例均被证实为非 GCB 细胞表型,并且均表达 IRF4/MUM1。2 例患者死于疾病,4 例患者在接受 R-CHOP 治疗后完全缓解且存活,2 例患者刚刚被诊断出。与 EBV 阴性组相比,没有可靠的形态学和免疫组织化学特征表明 EBV 阳性。因此,需要进行 EBER 原位杂交分析以确定“老年 EBV 阳性 DLBCL”。需要进一步研究以充分了解该疾病的细节,这可能会导致新的治疗方法。

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