Primary intestinal Epstein⇓Barr virus-associated natural killer/T-cell lymphoproliferative disorder: A disease mimicking inflammatory bowel disease

Zhujun Wang, Wenyan Zhang, Chengxin Luo, Min Zhu, Yu Zhen, Jingxi Mu, Yan Zhang, Renwei Hu, Yufang Wang, Zhonghui Wen, Qin Ouyang, Shuyuan Xiao, Hu Zhang

Research output: Contribution to journalArticlepeer-review

Abstract

Background and Aims: 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. Methods: 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. Results: 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 positiveT-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. Conclusions: PIEBV+ NK/T-LPD may be difficult to differentiate from IBD orTB owing to overlapping endoscopic and pathological findings. Early identification of EBV reactivation in tissue samples is essential for the accurate diagnosis.

Original languageEnglish
Pages (from-to)896-904
Number of pages9
JournalJournal of Crohn's and Colitis
Volume12
Issue number8
DOIs
Publication statusPublished - 2018 Jul 30
Externally publishedYes

Keywords

  • Differential diagnosis
  • Inflammatory bowel disease
  • Primary intestinal EBV-associated NK/T-cell lymphoproliferative disorders

ASJC Scopus subject areas

  • Gastroenterology

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