Extranodal NK/T-cell lymphoma, nasal type, had a suspicious background of presentation of chronic active EB virus infection

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  • Shirahase Akiko
    Department of Pediatrics, Shiga University of Medical Science Department of Pediatrics, Omihachiman Community Medical Center
  • Matsukawa Yukihiro
    Department of Pediatrics, Shiga University of Medical Science
  • Ikeda Yuhachi
    Department of Pediatrics, Shiga University of Medical Science
  • Sakamoto Kenichi
    Department of Pediatrics, Shiga University of Medical Science
  • Taga Takashi
    Department of Pediatrics, Shiga University of Medical Science
  • Maruo Yoshihiro
    Department of Pediatrics, Shiga University of Medical Science

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  • 節外性NK/Tリンパ腫発症の背景に慢性活動性EBウイルス感染症の存在が示唆された1例

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Abstract

<p>Chronic active Epstein–Barr virus (EBV) infection (CAEBV) has an indolent clinical course and a highly variable presentation, with symptoms including fever, lymphadenopathy, and hepatosplenomegaly. The clonality of CAEBV, which frequently involves T and/or natural killer (NK) cells, has been suggested by the monoclonal patterns of the virus. Extranodal NK/T-cell lymphoma, nasal type (ENKL), is an EBV-associated lymphoma and is most common among people in their 40s to 50s, but rare in children. A 12-year-old boy was diagnosed as having ENKL, which was complicated owing to a high number of EBV-DNA copies and EBV titer in the peripheral blood. ENKL resolved with radiochemotherapy, but the number of EBV-DNA copies did not decrease. Thus, ENKL with CAEBV as a background disease was diagnosed and hematopoietic stem-cell transplantation was performed. Complete remission of CAEBV was achieved. Thus, when diagnosing childhood-onset ENKL, CAEBV should always be considered as a possible underlying disease.</p>

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