Dziadzio Magdalena, Chee Ronnie, McNamara Christopher, Deheragoda Maesha, Wagner Thomas, Seneviratne Suranjith L
Department of Immunology, Royal Free Hospital, London, UK.
BMJ Case Rep. 2013 Aug 21;2013:bcr2013009721. doi: 10.1136/bcr-2013-009721.
Idiopathic CD4 lymphocytopenia (ICL) is a rare immunodeficiency disorder. We describe a 49-year-old woman with a history of ICL who developed hepatic Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL). ICL was diagnosed at a time of her presentation with varicella-zoster virus (VZV) meningoencephalitis and chorioretinitis. Her CD4 count subsequently improved but remained at the lower limits of the normal range. Five years later she presented with cough, fever and night-sweat. She was found to have multiple liver nodules on MRI, fluorodeoxyglucose (FDG) avid on the positron emission tomography (PET) CT, histologically defined as DLBCL, EBV positive and of non-germinal centre type. To our knowledge this is the first reported case of EBV-positive DLBCL localised to the liver in the context of ICL. EBV-positive DLBCL typically occurs in immunocompromised individuals. The corticosteroid therapy she received for VZV meningoencephalitis may have contributed to the EBV reactivation with subsequent EBV-driven malignant transformation of B-cells.
特发性CD4淋巴细胞减少症（ICL）是一种罕见的免疫缺陷疾病。我们描述了一名49岁患有ICL病史的女性，她发展为肝 Epstein-Barr病毒（EBV）阳性弥漫性大B细胞淋巴瘤（DLBCL）。ICL在她因水痘带状疱疹病毒（VZV）脑膜脑炎和脉络膜视网膜炎就诊时被诊断出来。她的CD4计数随后有所改善，但仍处于正常范围的下限。五年后，她出现咳嗽、发热和盗汗。MRI检查发现她有多个肝脏结节，正电子发射断层扫描（PET）CT显示氟脱氧葡萄糖（FDG）摄取增加，组织学诊断为DLBCL，EBV阳性且为非生发中心型。据我们所知，这是首例在ICL背景下报告的局限于肝脏的EBV阳性DLBCL病例。EBV阳性DLBCL通常发生在免疫功能低下的个体中。她因VZV脑膜脑炎接受的皮质类固醇治疗可能促成了EBV的重新激活，随后导致B细胞发生EBV驱动的恶性转化。
