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免疫功能正常患者肛管发生的爱泼斯坦-巴尔病毒阳性浆母细胞淋巴瘤

Epstein-Barr Virus-Positive Plasmablastic Lymphoma Arising in the Anal Canal of an Immunocompetent Patient.

作者信息

Al-Majmuei Abdulrahman, Al-Naqeeb Abdullah, Fakhri Farah, Murray Paul G, Aljuffairi Eman

机构信息

Department of Pathology, School of Medicine, Royal College of Surgeons in Ireland - Bahrain, Muharraq, BHR.

Pathology and Laboratory Medicine, King Hamad University Hospital, Muharraq, BHR.

出版信息

Cureus. 2025 Aug 11;17(8):e89785. doi: 10.7759/cureus.89785. eCollection 2025 Aug.

DOI:10.7759/cureus.89785
PMID:40937257
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12421462/
Abstract

This case report describes a 57-year-old immunocompetent male diagnosed with Epstein-Barr virus (EBV)-positive plasmablastic lymphoma (PBL), a rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL), presenting in the anal canal. PBL is most commonly seen in immunocompromised patients, particularly those with a human immunodeficiency virus (HIV) infection; its presence in immunocompetent individuals is exceedingly rare. The patient presented with persistent anal pain, prompting a comprehensive diagnostic workup. Histopathological and immunohistochemical analysis confirmed PBL, with a high proliferation index and EBV-encoded RNA (EBER) positivity. The patient initially responded to dose-adjusted rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-R-EPOCH) chemotherapy, with a reduction in mass size and metabolic activity. However, the disease relapsed, requiring further treatment with ifosfamide, carboplatin, and etoposide (ICE) chemotherapy, followed by consolidation with autologous stem cell transplantation (ASCT), after which the patient remained in remission for six months. This report not only discusses the clinical and pathological findings but also explores the interplay between EBV and the immune system in tumor development. The case underscores the need for heightened clinical suspicion and the future potential for immunotherapeutic and EBV-targeted therapies for patients with EBV-positive PBL.

摘要

本病例报告描述了一名57岁免疫功能正常的男性,被诊断为感染爱泼斯坦-巴尔病毒(EBV)的浆母细胞淋巴瘤(PBL),这是弥漫性大B细胞淋巴瘤(DLBCL)的一种罕见且侵袭性亚型,病变位于肛管。PBL最常见于免疫功能低下的患者,尤其是那些感染人类免疫缺陷病毒(HIV)的患者;在免疫功能正常的个体中极为罕见。该患者因持续性肛门疼痛就诊,从而进行了全面的诊断检查。组织病理学和免疫组织化学分析确诊为PBL,具有高增殖指数和EBV编码RNA(EBER)阳性。患者最初对剂量调整的利妥昔单抗、依托泊苷、泼尼松、长春新碱、环磷酰胺和多柔比星(DA-R-EPOCH)化疗有反应,肿块大小和代谢活性降低。然而,疾病复发,需要用异环磷酰胺、卡铂和依托泊苷(ICE)化疗进一步治疗,随后进行自体干细胞移植(ASCT)巩固治疗,此后患者缓解了六个月。本报告不仅讨论了临床和病理结果,还探讨了EBV与免疫系统在肿瘤发生中的相互作用。该病例强调了提高临床怀疑的必要性以及EBV阳性PBL患者免疫治疗和EBV靶向治疗的未来潜力。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/7ed5a2f98ab3/cureus-0017-00000089785-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/85de17271966/cureus-0017-00000089785-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/d83eb512edd7/cureus-0017-00000089785-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/913cafcd7686/cureus-0017-00000089785-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/cb2e6467b433/cureus-0017-00000089785-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/7ed5a2f98ab3/cureus-0017-00000089785-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/85de17271966/cureus-0017-00000089785-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/d83eb512edd7/cureus-0017-00000089785-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/913cafcd7686/cureus-0017-00000089785-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/cb2e6467b433/cureus-0017-00000089785-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac4/12421462/7ed5a2f98ab3/cureus-0017-00000089785-i05.jpg

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