Current research on chronic active Epstein-Barr virus infection in Japan

Shigeyoshi Fujiwara, Hiroshi Kimura, Ken Ichi Imadome, Ayako Arai, Eiichi Kodama, Tomohiro Morio, Norio Shimizu, Hiroshi Wakiguchi

Research output: Contribution to journalReview articlepeer-review

69 Citations (Scopus)


Epstein-Barr virus (EBV) infection is usually asymptomatic and persists lifelong. Although EBV-infected B cells have the potential for unlimited proliferation, they are effectively removed by the virus-specific cytotoxic T cells, and EBV-associated lymphoproliferative disease develops only in immunocompromised hosts. Rarely, however, individuals without apparent immunodeficiency develop chronic EBV infection with persistent infectious mononucleosis-like symptoms. These patients have high EBV-DNA load in the peripheral blood and systemic clonal expansion of EBV-infected T cells or natural killer (NK) cells. Their prognosis is poor with life-threatening complications including hemophagocytic lymphohistiocytosis, organ failure, and malignant lymphomas. The term "chronic active EBV infection" (CAEBV) is now generally used for this disease. The geographical distribution of CAEBV is markedly uneven and most cases have been reported from Japan and other East Asian countries. Here we summarize the current understanding of CAEBV and describe the recent progress of CAEBV research in Japan.

Original languageEnglish
Pages (from-to)159-166
Number of pages8
JournalPediatrics International
Issue number2
Publication statusPublished - 2014 Apr


  • EBV-associated T/NK-cell lymphoproliferative disease
  • EBV-associated hemophagocytic lymphohistiocytosis
  • Epstein-Barr virus
  • chronic active EBV infection
  • flow-cytometric in situ hybridization
  • hydroa vacciniforme
  • hypersensitivity to mosquito bites
  • mouse model

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


Dive into the research topics of 'Current research on chronic active Epstein-Barr virus infection in Japan'. Together they form a unique fingerprint.

Cite this