EB 病毒 (EBV) 阳性原发性皮肤边缘区淋巴瘤 (PCMZL) 并不常见，随后的转化也很少见。我们报告了一名 EBV 阳性 PCMZL 患者随后转化为浆母细胞淋巴瘤，并回顾了转化 PCMZL 的文献以评估临床和病理学特征。在我们描述的病例中，患者出现多灶性 PCMZL，发生大 B 细胞转化并伴有浆细胞分化，随后发生浆母细胞转化 (PBL)，尽管接受了多种治疗，但最终还是因疾病进展而死亡。既往病史对于银屑病关节炎很重要（之前接受过多种免疫调节治疗）。淋巴瘤和非受累骨髓具有相同的体细胞 DNMT3A 和 TET2 突变，表明克隆相关性以及与克隆造血 (CH) 的关联。 18 例病例符合该队列（17 例来自文献和本文报告的病例）。 18 例发生转化的 PCMZL 病例中近一半死于疾病进展（44%）。转化病例更常见于初次诊断时部位超过 2 个的患者。对 5 名患者进行了 EBV 评估，其中 3 名呈阳性（全部死于疾病）。两名进行 NGS 研究的患者显示 TET2 和 DNMT3A 突变。EBV 阳性 PCMZL 的转化似乎是一个不良的预后指标，我们报告的病例是第一个明确的转化为 PBL 的病例，怀疑由骨髓性 CH 引起。© 作者（ s) 2024 年。由牛津大学出版社代表美国临床病理学会出版。版权所有。如需商业重复使用，请联系 reprints@oup.com 获取转载和转载的翻译权。所有其他权限都可以通过我们网站文章页面上的权限链接通过我们的 RightsLink 服务获得 - 如需更多信息，请联系journals.permissions@oup.com。

Epstein-Barr Virus (EBV) positive primary cutaneous marginal zone lymphoma (PCMZL) is uncommon and subsequent transformation is rare.We report a patient with EBV positive PCMZL with subsequent transformation to plasmablastic lymphoma and review the literature for transformed PCMZL to assess clinical and pathologic characteristics. In the case we describe, the patient presented with multifocal PCMZL, developed large B cell transformation with plasmacytic differentiation, followed by plasmablastic transformation (PBL), and ultimately died of disease progression despite multiple lines of therapy. Past history was significant for psoriatic arthritis (multiple prior lines of immunomodulatory therapy). The lymphomas and non-involved bone marrow share the same somatic DNMT3A and TET2 mutations, suggesting clonal relatedness and an association with clonal hematopoiesis (CH).Eighteen cases complied the cohort (seventeen cases from the literature and the case reported herein). Nearly half of the eighteen cases of PCMZL with transformation died of progressive disease (44%). Transformed cases were more commonly seen in patients with >2 sites at initial diagnosis. EBV was assessed in 5 patients, 3 were positive (all died of disease). Two patients with NGS studies demonstrated TET2 and DNMT3A mutations.Transformation of EBV positive PCMZL appears to be a poor prognostic indicator, with our reported case being the first well defined case transformed to PBL, suspected to arise from myeloid-CH.© The Author(s) 2024. Published by Oxford University Press on behalf of American Society for Clinical Pathology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.