关键词: EBV‐positive lymphoproliferative disorder Epstein–Barr virus chronic active Epstein–Barr virus infection‐associated enteritis inflammatory bowel disease misdiagnosis

来  源:   DOI:10.1002/ccr3.8919   PDF(Pubmed)

Abstract:
Chronic active Epstein-Barr virus (EBV) infection-associated enteritis (CAEAE) in nonimmunodeficient individuals is rare. To report a case of CAEAE, relevant articles were searched through databases. The clinical manifestations, endoscopic findings, strategies of treatment, prognoses, and follow-up results of CAEAE patients were analyzed. Including this report, seven citations in the literature provide descriptions of 27 cases of CAEAE. There were 21 males and six females, with a mean age of 40 years. The main clinical manifestations were fever (25/27), abdominal pain (14/27), diarrhea (16/27), hematochezia or bloody stools (13/27), and decreased hemoglobin and red blood cell counts in routine blood tests (14/27). Elevations in inflammatory markers, white blood cell (WBC) counts, and C-reactive protein (CRP) were common. Coagulation was often abnormal. Histopathology confirmed EBV-encoded small nuclear RNA (EBER) in the affected tissue via in situ hybridization. The average serum EBV DNA load was 6.3 × 10^5 copies/mL. All patients had varying degrees of intestinal ulcers endoscopically, and the ulcers and pathology were uncharacterized and misdiagnosed mostly as inflammatory bowel disease (IBD). The course of the disease was progressive and later complicated by intestinal bleeding, intestinal perforation, septic shock, and a high rate of emergency surgery. However, the conditions of the patients often did not improve after surgery, and some patients soon died due to reperforation or massive hematochezia. Hormone and antiviral treatment had no obvious effect. There was a significant difference in surgical and nonsurgical survival (p < 0.05). The proportion of patients who died within 6 months was as high as 63.6% (7/11). CAEAE belongs to a group of rare, difficult conditions, has an insidious clinical course, has a high case fatality rate, and may later develop into EBV-positive lymphoproliferative disorder (EBV-LPD), which in turn leads to carcinogenesis. Clinicians should raise awareness that in patients with multiple ulcers in the intestine of unknown etiology, attention should be paid to EBV serology, and histology to make the diagnosis as early as possible.
摘要:
在非免疫缺陷个体中,慢性活动性EB病毒(EBV)感染相关性肠炎(CAEAE)很少见。报告一例CAEAE,通过数据库搜索相关文章。临床表现,内镜检查结果,治疗策略,预后,并对CAEAE患者的随访结果进行分析。包括这份报告,文献中的7篇引文对27例CAEAE进行了描述。有21名男性和6名女性,平均年龄40岁.主要临床表现为发热(25/27),腹痛(14/27),腹泻(16/27),便血或血便(13/27),血常规检查中血红蛋白和红细胞计数下降(14/27)。炎症标志物升高,白细胞(WBC)计数,C反应蛋白(CRP)是常见的。凝血常异常。组织病理学通过原位杂交证实了受影响组织中EBV编码的小核RNA(EBER)。平均血清EBVDNA载量为6.3×10^5拷贝/mL。所有患者在内镜下都有不同程度的肠溃疡,溃疡和病理特征不明,误诊为炎症性肠病(IBD)。病程进展,后来并发肠出血,肠穿孔,感染性休克,急诊手术率很高。然而,手术后患者的状况往往没有改善,一些患者很快因再穿孔或大量便血死亡。激素和抗病毒治疗均无明显效果。手术和非手术生存率有显著差异(p<0.05)。6个月内死亡的患者比例高达63.6%(7/11)。CAEAE属于一组罕见的,困难的条件,有一个阴险的临床过程,病死率很高,并可能后来发展为EBV阳性淋巴增生性疾病(EBV-LPD),进而导致致癌作用。临床医生应提高对病因不明的肠道多发溃疡患者的认识,应注意EBV血清学,和组织学尽可能早地做出诊断。
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