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首页> 外文期刊>BMC Gastroenterology >Chronic active EBV infection in refractory enteritis with longitudinal ulcers with a cobblestone appearance: an autopsied case report
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Chronic active EBV infection in refractory enteritis with longitudinal ulcers with a cobblestone appearance: an autopsied case report

机译：耐火肠炎慢性活性EBV感染，具有鹅卵石外观的纵向溃疡：尸检案例报告

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Chronic active Epstein–Barr virus infection (CAEBV) is defined as Epstein–Barr virus (EBV)-positive T/NK cell-related neoplasia, and its major clinical symptom is systemic inflammation presenting as infectious mononucleocytosis, whereas enteritis and diarrhea are minor clinical symptoms. The complex mixture of tumorigenic processes of EBV-positive cells and physical symptoms of systemic inflammatory disease constitutes the varied phenotypes of CAEBV. Herein, we describe a case of CAEBV that was initially diagnosed as Crohn’s disease (CD) based on ileal ulcers and clinical symptoms of enteritis. A 19-year-old woman complained of abdominal pain and fever. Blood examination showed normal blood cell counts without atypical lymphocyte but detected modest inflammation, hypoalbuminemia, slight liver dysfunction, and evidence of past EBV infection. The esophagogastroduodenoscopic findings were normal. However, colonoscopy revealed a few small ulcers in the terminal ileum. The jejunum and ileum also exhibited various forms of ulcers, exhibiting a cobblestone appearance, on capsule endoscopy. Based on these clinical findings, she was strongly suspected with CD. In the course of treatment by steroid and biologics for refractory enteritis, skin ulcers appeared about 50?months after her initial hospital visit. Immunohistology of her skin biopsy revealed proliferation of EBV-encoded small RNA (EBER)-positive atypical lymphocytes. We retrospectively assessed her previous ileal ulcer biopsy before treatment and found many EBER-positive lymphocytes. Blood EBV DNA was also positive. Therefore, she was diagnosed with extranodal NK/T-cell lymphoma with CAEBV-related enteritis rather than CD. She was treated with cyclosporine and prednisolone combination therapy for CAEBV-related systemic inflammation and chemotherapy for malignant lymphoma. Unfortunately, her disease continued to progress, leading to multiple organ failure and death at the age of 23?years. Clinicians need to remember the possibility of CAEBV as a differential diagnosis of refractory enteritis. Enteritis with intestinal ulcer is a rare symptom of CAEBV, and it is impossible to acquire a definitive diagnosis by ulcer morphology only. In cases where the possibility of CAEBV remains, tissue EBVR expression should be checked by in situ hybridization and blood EBV DNA.
机译：慢性活性Epstein-BART病毒感染（CAEBV）被定义为Epstein-BART病毒（EBV） - 阳性T / NK细胞相关的肿瘤，其主要临床症状是呈现为传染性单核霉素的全身炎症，而肠炎和腹泻是轻微的临床症状。 EBV阳性细胞的致瘤过程的复杂混合物和全身炎症疾病的物理症状构成了Caebv的变化表型。在此，我们描述了Caebv的情况，该病例最初被诊断为基于肠炎溃疡和肠炎的临床症状的CROHN疾病（CD）。一个19岁的女性抱怨腹痛和发烧。血液检查显示常规血细胞计数没有非典型淋巴细胞，但检测到适度的炎症，低恶蛋白血症，轻微肝功能障碍以及过去EBV感染的证据。食管冈古代透视结果正常。然而，结肠镜检查在终端期间揭示了一些小溃疡。 Jejunum和Eleum还表现出各种形式的溃疡，在胶囊内窥镜检查中表现出鹅卵石外观。基于这些临床发现，她强烈怀疑CD。在难治性肠炎的类固醇和生物学的治疗过程中，皮肤溃疡出现在初始医院访问后约50个月。她的皮肤活检的免疫组织学揭示了EBV编码的小RNA（EBER） - 阳性非典型淋巴细胞的增殖。我们回顾性地评估了治疗前以前的髂骨溃疡活检，发现了许多Eber阳性淋巴细胞。血液EBV DNA也是阳性的。因此，她被诊断出患有Caebv相关的肠炎而不是Cd的外胚层NK / T细胞淋巴瘤。她对Caebv相关的全身炎症和化疗进行了环孢菌素和泼尼松组合治疗治疗恶性淋巴瘤。不幸的是，她的疾病继续进展，导致23岁的器官失败和死亡。临床医生需要记住CAEBV作为难肠炎的差异诊断的可能性。肠炎具有肠道溃疡是Caebv的罕见症状，只能通过溃疡形态获得最终的诊断。在Caebv的可能性仍然存在的情况下，应通过原位杂交和血液EBV DNA检查组织EBVR表达。

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