title: Clinicopathological analysis of 12 patients with Epstein-Barr virus-positive primary intestinal T/natural killer-cell lymphoma (EBV+ITNKL).
abstract: OBJECTIVE
  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.
  
  
  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.
authors.affiliation: Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.|Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.
authors.forename: Lei-Ming
authors.initials: LM
authors.lastname: Hu
authors.affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Takata
authors.affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
authors.forename: Tomoko
authors.initials: T
authors.lastname: Miyata-Takata
authors.affiliation: Department of Molecular Diagnostics, Nagano Prefectural Suzaka Hospital, Suzaka, Japan.
authors.forename: Naoko
authors.initials: N
authors.lastname: Asano
authors.affiliation: Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute, Japan.
authors.forename: Emiko
authors.initials: E
authors.lastname: Takahashi
authors.affiliation: Department of Hematology, Ogaki Municipal Hospital, Ogaki, Japan.
authors.forename: Katsuya
authors.initials: K
authors.lastname: Furukawa
authors.affiliation: Department of Pathology, Kurume University School of Medicine, Kurume, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Miyoshi
authors.affiliation: Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.|Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Satou
authors.affiliation: Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.
authors.forename: Kei
authors.initials: K
authors.lastname: Kohno
authors.affiliation: Department of Hematology, Ogaki Municipal Hospital, Ogaki, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kosugi
authors.affiliation: Department of Hematology and Cell Therapy, Aichi Cancer Center, Nagoya, Japan.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Kinoshita
authors.affiliation: Department of Endoscopy, Nagoya University Hospital, Nagoya, Japan.
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Hirooka
authors.affiliation: Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Hidemi
authors.initials: H
authors.lastname: Goto
authors.affiliation: Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
authors.affiliation: Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.
authors.forename: Seiichi
authors.initials: S
authors.lastname: Kato
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058527:Enteropathy-Associated T-Cell Lymphoma / Q000401:mortality / Q000473:pathology* / Q000821:virology*; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000453:epidemiology; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D055611:Natural Killer T-Cells / Q000473:pathology*
keywords: chronic active EBV infection; intestinal T/NK cell lymphoma; intra-epithelial lymphocytosis T cell receptor