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T/NK cell type chronic active Epstein–Barr virus disease in adults: an underlying condition for Epstein–Barr virus-associated T/NK-cell lymphoma
  1. Yasushi Isobe1,
  2. Nanae Aritaka1,
  3. Yasuhiro Setoguchi2,
  4. Yoshinori Ito3,
  5. Hiroshi Kimura4,
  6. Yasuharu Hamano1,
  7. Koichi Sugimoto1,
  8. Norio Komatsu1
  1. 1Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan
  2. 2Department of Respiratory Medicine, Tokyo Medical University, Tokyo, Japan
  3. 3Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
  4. 4Department of Virology, Nagoya University Graduate School of Medicine, Nagoya, Japan
  1. Correspondence to Dr Yasushi Isobe, Department of Hematology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan; yisobe{at}juntendo.ac.jp

Abstract

A chronic infectious mononucleosis-like illness caused by Epstein–Barr virus (EBV) is called ‘chronic active EBV disease’, which is defined as an EBV-associated lymphoproliferative disease. This lymphoproliferative disease is rare and predominantly occurs in Japanese children. Between 1998 and 2010, seven adult-onset cases (aged 20–45 years, median 39 years) were identified, which initially presented with inflammatory diseases, including hepatitis, interstitial pneumonitis, uveitis, nephritis and hypersensitivity to mosquito bites. They showed an EBV viral load in the peripheral blood and evidence of EBV infection of T or natural killer (NK) cells. Five cases (71.4%) developed EBV-positive T/NK-cell lymphoma/leukaemia at a median of 5 years (range 1–7 years) after the diagnosis. Although l-asparaginase-containing chemotherapy was effective for the lymphomas, only allogeneic haematopoietic cell transplantation eradicated EBV-infected cells. This observation indicates that persistent EBV infection of T or NK cells defines a distinct disease entity, which provides an underlying condition for EBV-positive T/NK-cell lymphoma/leukaemia.

  • Allogeneic haematopoietic cell transplantation
  • chronic active EBV disease
  • EBV
  • genetics
  • l-asparaginase
  • leukaemia
  • lung
  • lung cancer
  • lymphoma
  • T/NK cell type

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.

  • Ethics approval This study was approved by Institutional Review Board at Juntendo University.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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