Chen Nan, Perez Mike, Mims Martha
Baylor College of Medicine, 1 Baylor Plaza, Houston, TX 77030, USA.
Case Rep Hematol. 2019 Oct 7;2019:7962485. doi: 10.1155/2019/7962485. eCollection 2019.
EBV (Epstein-Barr virus) viremia causes immune dysregulation through various mechanisms, and we are understanding more that mutations in B, T, and NK (natural killer) cell signaling pathways allow EBV complications such as HLH (hemophagocytic lymphohistiocytosis) and lymphomas to arise. Here, we report a 20-year-old previously healthy, HIV- (human immunodeficiency virus-) negative male who presented with fevers, sore throat, and lymphadenopathy (LAD). He was found to have EBV viremia, pancytopenia, and elevated LFTs (liver function tests) suspicious for HLH. Bone marrow biopsy and elevated IL-2 (interleukin) receptor confirmed this diagnosis. Additionally, gastric biopsy confirmed diagnosis of plasmablastic lymphoma (PBL), a rare, aggressive HIV- and EBV-associated lymphoma. Both bone marrow and gastric biopsy showed evidence of EBV. Patients with EBV complications should have a rigorous workup to characterize the full extent of immune dysregulation including genetic testing at a high-volume center.
爱泼斯坦-巴尔病毒（EBV）血症通过多种机制导致免疫失调，并且我们越来越多地了解到，B、T和自然杀伤（NK）细胞信号通路中的突变会引发诸如噬血细胞性淋巴组织细胞增生症（HLH）和淋巴瘤等EBV相关并发症。在此，我们报告一名20岁、既往健康、人类免疫缺陷病毒（HIV）阴性的男性，他出现发热、咽痛和淋巴结病（LAD）。他被发现患有EBV血症、全血细胞减少以及肝功能检查（LFTs）升高，怀疑患有HLH。骨髓活检和白细胞介素-2（IL-2）受体升高证实了这一诊断。此外，胃活检确诊为浆母细胞淋巴瘤（PBL），这是一种罕见的、侵袭性的与HIV和EBV相关的淋巴瘤。骨髓和胃活检均显示有EBV存在的证据。患有EBV相关并发症的患者应进行严格检查，以全面评估免疫失调的程度，包括在大型中心进行基因检测。
