慢性活動性EBウイルス病の臨床的寛解中に発症した節外性NK/T細胞リンパ腫の1例

磯部 清孝; 杉山 正仲; 田尾 佳代子; 荒川 歩; 前島 亜希子; 金田 朋也; 福田 隆浩; 今留 謙一; 小川 千登世

doi:10.11412/jspho.62.74

Case Report

A case of extranodal NK/T-cell lymphoma during clinical remission of chronic active Epstein–Barr virus disease

Kiyotaka Isobe, Masanaka Sugiyama, Kayoko Tao, Ayumu Arakawa, Akiko Maeshima, Tomoya Kaneda, Takahiro Fukuda, Ken-Ichi Imadome, Chitose Ogawa

Author information

Kiyotaka Isobe
Department of Pediatric Oncology, National Cancer Center Hospital
Masanaka Sugiyama
Department of Pediatric Oncology, National Cancer Center Hospital
Kayoko Tao
Department of Pediatric Oncology, National Cancer Center Hospital
Ayumu Arakawa
Department of Pediatric Oncology, National Cancer Center Hospital
Akiko Maeshima
Department of Diagnostic Pathology, National Cancer Center Hospital
Tomoya Kaneda
Department of Radiation Oncology, National Cancer Center Hospital
Takahiro Fukuda
Department of Hematopoietic Stem Cell Transplantation, National Cancer Center Hospital
Ken-Ichi Imadome
Department of Advanced Medicine for Infections, National Center for Child Health and Development
Chitose Ogawa
Department of Pediatric Oncology, National Cancer Center Hospital

Keywords: chronic active EBV disease, extranodal NK/T-cell lymphoma, skin ulceration, SMILE therapy, allogeneic hematopoietic stem cell transplantation

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2025 Volume 62 Issue 1 Pages 74-79

DOI https://ntrd-jsjc-gov-cn-s-1416.res.gxlib.org.cn:443/rwt/1416/https/MSYXTLUQPJUB/10.11412/jspho.62.74

Details

Abstract

Studies have yet to report on extranodal NK/T-cell lymphoma (ENKL) after remission of chronic active Epstein–Barr virus (CAEBV) disease and specify its treatment. Here, we report a case of ENKL in the subcutaneous right lower leg during clinical remission of CAEBV that was successfully treated with SMILE therapy and allogeneic hematopoietic stem cell transplantation (allo-HSCT). A 14-year-old boy was diagnosed with CAEBV and underwent allo-HSCT at 10 years of age. Recently, he developed a subcutaneous mass in the right lower leg and skin ulcers, which were biopsied and diagnosed as ENKL. The patient exhibited partial metabolic response after two courses of SMILE therapy. We performed allo-HSCT after an additional SMILE therapy course. The patient is alive and disease-free 19 months post-transplantation, indicating that SMILE therapy is effective for ENKL that develops subcutaneously after CAEBV remission and allo-HSCT may be effective as a consolidation therapy for long-term survival.

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