Clinicopathological analysis of 12 patients with Epstein–Barr virus-positive primary intestinal T/natural killer-cell lymphoma (EBV+ ITNKL)

Lei Ming Hu, Katsuyoshi Takata, Tomoko Miyata-Takata, Naoko Asano, Emiko Takahashi, Katsuya Furukawa, Hiroaki Miyoshi, Akira Satou, Kei Kohno, Hiroshi Kosugi, Tomohiro Kinoshita, Yoshiki Hirooka, Hidemi Goto, Shigeo Nakamura, Seiichi Kato

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14 Citations (Scopus)


Aims: Epstein–Barr virus-positive (EBV+) intestinal T/natural killer (NK) cell lymphoma (ITNKL) is an uncommon tumour with an extremely aggressive clinical behaviour. However, the clinicopathological characteristics of this tumour, including T cell receptor (TCR) phenotype and the patient's background, remain unknown. The aim of this study was to elucidate the detailed clinicopathological profile of EBV+ ITNKL. Methods and results: We enrolled 12 patients with EBV+ ITNKL without nasal involvement into the study. All patients were characterized by involvement of the small intestine with concurrent lesions of the large intestine in two patients. Seven patients (58%) had Lugano stages IIE/IV disease and eight (67%) were categorized as high–intermediate/high-risk according to the Prognostic Index for PTCL (PIT). Three patients (25%) with an age of onset of less than 50 years had chronic active EBV infection (CAEBV). Five CD56-positive patients (42%) had a poorer prognosis than those without CD56 expression (P = 0.008). NK cell-type lymphoma defined by the absence of any TCR expression or clonal TCR-γ rearrangement was found in six patients (50%). Interestingly, EBV+ intra-epithelial lymphocytosis was observed in one case with a background of CAEBV. Conclusions: This study is the first to shed light on the significant heterogeneity of EBV+ ITNKL and its relationship with CAEBV, especially in patients younger than 50 years of age. These observations will provide a guide for diagnostic and therapeutic approaches in routine practice.

Original languageEnglish
Pages (from-to)1052-1063
Number of pages12
Issue number7
Publication statusPublished - 06-2017
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Histology


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