一名免疫功能正常女性的 Epstein-Barr 病毒阳性浆细胞瘤:病例报告。

IF 0.9 Q4 HEMATOLOGY
Hiyo Okuno, Motoha Miura, Naoki Oishi, Megumi Koshiishi-Yamada, Fumihiko Tanioka, Keita Kirito, Tetsuo Kondo
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引用次数: 0

摘要

浆细胞瘤的定义是形成单发骨性或髓外肿瘤的浆细胞肿瘤,没有骨髓瘤或浆细胞肿瘤相关器官损伤的证据。爱泼斯坦-巴氏病毒(EBV)与各种B细胞肿瘤有关,尤其是在免疫失调的患者中;然而,浆细胞瘤通常对EBV呈阴性反应。本文介绍了一例免疫功能正常女性胸骨浆细胞瘤 EBV 阳性病例。一名 76 岁女性,无免疫缺陷,胸前壁出现肿瘤。影像学分析显示,鬃骨处有一个 6.3 厘米大小的肿瘤,于是进行了针刺活检。骨中的肿瘤由弥漫增生的浆细胞组成,具有偏心核和核周晕。通过免疫组化和原位杂交,肿瘤细胞为 CD20-、CD3-、CD138+、κ+、λ-、EBER+,Ki67 标记指数约为 20%。随后的研究发现血清中存在IgG κ单克隆蛋白,但没有证据表明存在浆细胞肿瘤相关的器官损伤,如高钙血症、贫血或肾功能障碍。在骨髓形态学和流式细胞术研究中未发现浆细胞肿瘤。因此,诊断结果为 EBV 阳性浆细胞瘤。患者接受了局部放射治疗,病情完全缓解。EBV阳性浆细胞瘤在免疫功能正常的患者中非常罕见,应与浆细胞性淋巴瘤仔细鉴别,后者是另一种EBV阳性肿瘤,具有浆细胞表型和侵袭性临床病程。这个病例也提出了一个重要问题:"在诊断浆细胞肿瘤时何时进行 EBER 原位杂交?
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Epstein-Barr virus-positive plasmacytoma in an immunocompetent female: A case report.

Plasmacytoma is defined as a plasma cell neoplasm forming a solitary osseous or extramedullary tumor without evidence of myeloma or organ damage related to a plasma cell neoplasm. Epstein-Barr virus (EBV) is associated with various B-cell neoplasms, particularly in patients with immune dysregulation; however, plasmacytoma is typically negative for EBV. Here, a case of EBV-positive sternal plasmacytoma in an immunocompetent female is presented. A 76-year-old female with no immunodeficiency presented with a tumor on the anterior thoracic wall. Imaging analysis revealed a 6.3 cm-sized tumor at the manubrium, and a needle biopsy was performed. The tumor in the bone was composed of a diffuse proliferation of plasmacytes with eccentric nuclei and a perinuclear halo. By immunohistochemistry and in situ hybridization, tumor cells were CD20-, CD3-, CD138+, κ+, λ-, EBER+, and the Ki67-labeling index was approximately 20%. Subsequent studies identified IgG κ monoclonal protein in serum but no evidence of plasma cell neoplasm-related organ damage, such as hypercalcemia, anemia, or renal dysfunction. No plasma cell neoplasm was detected in the bone marrow in the morphological and flowcytometric studies. Accordingly, the diagnosis was EBV-positive plasmacytoma. The patient was treated with local radiation therapy and achieved complete remission. EBV-positive plasmacytoma is rare in immunocompetent patients and should be carefully distinguished from plasmablastic lymphoma, another EBV-positive neoplasm with a plasma cell phenotype and an aggressive clinical course. This case also raises an important question: "when to perform EBER in situ hybridization in diagnosing plasma cell neoplasm?", which prompts further large case-series studies.

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来源期刊
CiteScore
2.00
自引率
6.70%
发文量
25
审稿时长
11 weeks
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