Sarwari Nawid M, Khoury Joseph D, Hernandez Cristhiam M Rojas
Department of Internal Medicine, University of Texas, Houston Health Sciences Center, Houston, TX USA.
Department of Hematopathology, University of Texas MD Anderson Cancer Center, Houston, TX USA.
BMC Hematol. 2016 Jul 19;16:19. doi: 10.1186/s12878-016-0059-3. eCollection 2016.
Chronic Epstein Barr virus (EBV) infection in an immunocompetent host has been described however it is not a common entity. It has been linked to many lymphoproliferative disorders and achieves such via many molecular mechanisms, some of which are poorly understood. In addition to infectious mononucleosis, the EBV is linked to various other hematological pathologies and autoimmune disorders.
We describe the case of an elderly immunocompetent female who presented with non-specific symptomatology, lymphadenopathy, cytopenias, elevated autoantibody titers and a crescent EBV viral load that were suggestive of a multisystemic inflammatory disease related to EBV. Extensive work up including multiple bone marrow biopsy and lymphoid tissue procedures ultimately led to the diagnosis of Hodgkin lymphoma.
EBV-related lymphomagenesis is complex and through the utilization of its nuclear antigens and latent membrane proteins the virus is able to shape the microenvironment to promote the various pathologies seen. Moreover, the diagnosis of EBV-associated lymphoproliferative disorders might be challenging when they present in immunocompetent individuals. Our case also represents an emphatic reminder for clinicians that spontaneous regression of lymphadenopathy is not exclusive of low-grade lymphoid malignancies.
免疫功能正常宿主中的慢性EB病毒(EBV)感染已有报道,但并不常见。它与许多淋巴增殖性疾病有关,并通过多种分子机制导致这些疾病,其中一些机制尚不清楚。除了传染性单核细胞增多症外,EBV还与各种其他血液病理学和自身免疫性疾病有关。
我们描述了一例老年免疫功能正常女性的病例,该患者表现为非特异性症状、淋巴结病、血细胞减少、自身抗体滴度升高以及EBV病毒载量呈新月形升高,提示与EBV相关的多系统炎症性疾病。包括多次骨髓活检和淋巴组织检查在内的广泛检查最终确诊为霍奇金淋巴瘤。
EBV相关的淋巴瘤发生是复杂的,通过利用其核抗原和潜伏膜蛋白,该病毒能够塑造微环境以促进所观察到的各种病理变化。此外,当EBV相关的淋巴增殖性疾病出现在免疫功能正常的个体中时,其诊断可能具有挑战性。我们的病例也强烈提醒临床医生,淋巴结病的自发消退并不排除低度淋巴恶性肿瘤。