International Journal of Hematology

, Volume 96, Issue 4, pp 443–449 | Cite as

Clonal expansion of Epstein–Barr virus (EBV)-infected γδ T cells in patients with chronic active EBV disease and hydroa vacciniforme-like eruptions

  • Taizo Wada
  • Akiko Toga
  • Yasuhisa Sakakibara
  • Tomoko Toma
  • Minoru Hasegawa
  • Kazuhiko Takehara
  • Tomonari Shigemura
  • Kazunaga Agematsu
  • Akihiro Yachie
Original Article

Abstract

Chronic active Epstein–Barr virus (EBV) disease (CAEBV) is a systemic EBV-positive lymphoproliferative disorder characterized by fever, lymphadenopathy, and splenomegaly. Patients with CAEBV may present with cutaneous symptoms, including hypersensitivity to mosquito bites and hydroa vacciniforme (HV)-like eruptions. HV is a rare photodermatosis characterized by vesicles and crust formation after exposure to sunlight, with onset in childhood, and is associated with latent EBV infection. While γδ T cells have recently been demonstrated to be the major EBV-infected cell population in HV, the immunophenotypic features of EBV-infected γδ T cells in CAEBV with HV-like eruptions or HV remain largely undetermined. We describe three patients with CAEBV whose γδ T cells were found to be the major cellular target of EBV. HV-like eruptions were observed in two of these patients. A clonally expanded subpopulation of γδ T cells that were highly activated and T cell receptor Vγ9- and Vδ2-positive cells was demonstrated in all patients. We also show that the clonally expanded γδ T cells infiltrated into the HV-like eruptions in one patient from whom skin biopsy specimens were available. These results suggest the pathogenic roles of clonally expanded γδ T cells infected by EBV in patients with CAEBV and HV-like eruptions.

Keywords

Epstein–Barr virus Clonal proliferation γδ T cells Hydroa vacciniforme 

Notes

Acknowledgments

We thank Ms Harumi Matsukawa and Ms. Shizu Kouraba for their excellent technical assistance. This work was supported by a grant from Takeda Science Foundation, Osaka; a Grant-in-Aid for Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology of Japan; and a grant from the Ministry of Health, Labour, and Welfare of Japan, Tokyo.

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© The Japanese Society of Hematology 2012

Authors and Affiliations

  • Taizo Wada
    • 1
  • Akiko Toga
    • 1
  • Yasuhisa Sakakibara
    • 1
  • Tomoko Toma
    • 1
  • Minoru Hasegawa
    • 2
  • Kazuhiko Takehara
    • 2
  • Tomonari Shigemura
    • 3
  • Kazunaga Agematsu
    • 4
  • Akihiro Yachie
    • 1
  1. 1.Department of Pediatrics, School of MedicineInstitute of Medical, Pharmaceutical and Health Sciences, Kanazawa UniversityKanazawaJapan
  2. 2.Department of Dermatology, School of MedicineInstitute of Medical, Pharmaceutical and Health Sciences, Kanazawa UniversityKanazawaJapan
  3. 3.Department of PediatricsShinshu University School of MedicineMatsumotoJapan
  4. 4.Department of Infection and Host DefenseShinshu University School of MedicineMatsumotoJapan

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