Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China.
Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
J Crohns Colitis. 2018 Jul 30;12(8):896-904. doi: 10.1093/ecco-jcc/jjy043.
Primary intestinal Epstein-Barr virus [EBV]-associated natural killer/T-cell lymphoproliferative disorder [PIEBV+ NK/T-LPD] is a rare clinical entity, which is difficult to differentiate from inflammatory bowel disease [IBD]. We present a series of Chinese patients with PIEBV+ NK/T-LPD to increase awareness among clinicians of this condition.
Patients diagnosed with PIEBV+ NK/T-LPD at West China hospital between 2014 and 2016 were included. Clinical and histopathological characteristics were reviewed, and key aspects of differential diagnosis were presented.
Twelve patients diagnosed with PIEBV+ NK/T-LPD were identified. Initial symptoms included intermittent fever [11/12 patients], abdominal pain [9/12], haematochezia [8/12], and diarrhoea [3/12]. Main endoscopic findings included multisegmental irregular, variable-sized ulcers, isolated giant ulcers, and diffuse inflammation. Colon and ileocaecum were mainly affected in 11 patients. The main PIEBV+ NK/T-LPD immunophenotypic profile of the infiltrating cells was CD3ε-positive NK/T cells characterised by positive T-cell intracellular antigen-1 and granzyme B, with CD5 deletion. In situ hybridisation was positive for EBV-encoded small RNAs 1/2 in all patients. Eleven patients were misdiagnosed with ulcerative colitis [4/11], Crohn's disease [4/11], or tuberculosis [TB, 3/11], owing to the similar endoscopic and histopathological features. The mean number of endoscopic procedures performed before reaching the diagnosis of PIEBV+ NK/T-LPD was 3.58; in four patients, the diagnosis was confirmed only after surgical resection following complications.
PIEBV+ NK/T-LPD may be difficult to differentiate from IBD or TB owing to overlapping endoscopic and pathological findings. Early identification of EBV reactivation in tissue samples is essential for the accurate diagnosis.
原发性肠道 EBV（Epstein-Barr virus）相关 NK/T 细胞淋巴瘤（PIEBV+NK/T-LPD）是一种罕见的临床实体，难以与炎症性肠病（IBD）相区分。我们呈现了一系列中国 PIEBV+NK/T-LPD 患者，旨在提高临床医生对这种疾病的认识。
纳入 2014 年至 2016 年期间在华西医院诊断为 PIEBV+NK/T-LPD 的患者。回顾了临床和组织病理学特征，并介绍了关键的鉴别诊断要点。
共确定了 12 例 PIEBV+NK/T-LPD 患者。初始症状包括间歇性发热[12 例中的 11 例]、腹痛[9 例中的 9 例]、血便[8 例中的 8 例]和腹泻[3 例中的 3 例]。主要内镜表现包括多节段不规则、大小不一的溃疡、孤立的巨大溃疡和弥漫性炎症。11 例患者主要累及结肠和回盲部。浸润细胞的主要 PIEBV+NK/T-LPD 免疫表型特征为 CD3ε 阳性 NK/T 细胞，特征为 T 细胞内抗原-1 和颗粒酶 B 阳性，CD5 缺失。所有患者的原位杂交均为 EBV 编码的小 RNA 1/2 阳性。由于内镜和组织病理学特征相似，11 例患者最初被误诊为溃疡性结肠炎[4/11]、克罗恩病[4/11]或结核病[3/11]。在明确诊断 PIEBV+NK/T-LPD 之前，平均进行了 3.58 次内镜检查；在 4 例患者中，仅在发生并发症后通过手术切除才得到确诊。
由于内镜和病理表现重叠，PIEBV+NK/T-LPD 可能难以与 IBD 或 TB 相区分。早期识别组织样本中的 EBV 再激活对于准确诊断至关重要。
