Development of Extranodal NK/T-cell Lymphoma Nasal Type in Cerebrum Following Epstein-Barr Virus-positive Uveitis

Ayano Imai; Hiroshi Takase; Ken-Ichi Imadome; Go Matsuda; Iichiro Ohnishi; Kouhei Yamamoto; Takumi Kudo; Yoji Tanaka; Taketoshi Maehara; Osamu Miura; Ayako Arai

doi:10.2169/internalmedicine.56.7573

CASE REPORTS

Development of Extranodal NK/T-cell Lymphoma Nasal Type in Cerebrum Following Epstein-Barr Virus-positive Uveitis

Ayano Imai, Hiroshi Takase, Ken-Ichi Imadome, Go Matsuda, Iichiro Ohnishi, Kouhei Yamamoto, Takumi Kudo, Yoji Tanaka, Taketoshi Maehara, Osamu Miura, Ayako Arai

Author information

Ayano Imai
Department of Ophthalmology, Tokyo Medical and Dental University, Japan
Hiroshi Takase
Department of Ophthalmology, Tokyo Medical and Dental University, Japan
Ken-Ichi Imadome
Division of Advanced Medicine for Virus Infections, National Research Institute for Child Health and Development, Japan
Go Matsuda
Division of Advanced Medicine for Virus Infections, National Research Institute for Child Health and Development, Japan
Iichiro Ohnishi
Department of Comprehensive Pathology, Tokyo Medical and Dental University, Japan
Kouhei Yamamoto
Department of Comprehensive Pathology, Tokyo Medical and Dental University, Japan
Takumi Kudo
Department of Functional Neurosurgery, Tokyo Medical and Dental University, Japan
Yoji Tanaka
Department of Functional Neurosurgery, Tokyo Medical and Dental University, Japan
Taketoshi Maehara
Department of Functional Neurosurgery, Tokyo Medical and Dental University, Japan
Osamu Miura
Department of Hematology, Tokyo Medical and Dental University, Japan
Ayako Arai
Department of Hematology, Tokyo Medical and Dental University, Japan

Keywords: extranodal NK/T-cell lymphoma nasal type, central nervous system, uveitis, Epstein-Barr virus

JOURNAL OPEN ACCESS

2017 Volume 56 Issue 11 Pages 1409-1414

DOI https://doi.org/10.2169/internalmedicine.56.7573

Details

Abstract

A 74-year-old woman developed bilateral uveitis with high Epstein-Barr virus (EBV) DNA load in the vitreous fluid without lymphoma cells. Four years after the onset, T2-weighted contrast-enhanced MRI revealed hyperintense lesions in the right occipital and parietal lobe. A biopsy resulted in the diagnosis of extranodal NK/T-cell lymphoma nasal type (ENKL). The repeat region of LMP1, an EBV gene, detected in the brain lesion was identical to that detected in the vitreous fluid. ENKL of the central nervous system is quite rare, and the pathogenesis has not been determined. The lymphoma in this case might have been closely associated with the EBV-positive uveitis.

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