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

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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.

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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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Authors and Affiliations

  1. Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, 13-1 Takaramachi, Kanazawa, 920-8641, Japan

    Taizo Wada, Akiko Toga, Yasuhisa Sakakibara, Tomoko Toma & Akihiro Yachie

  2. Department of Dermatology, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan

    Minoru Hasegawa & Kazuhiko Takehara

  3. Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan

    Tomonari Shigemura

  4. Department of Infection and Host Defense, Shinshu University School of Medicine, Matsumoto, Japan

    Kazunaga Agematsu

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  1. Taizo Wada
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  2. Akiko Toga
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  3. Yasuhisa Sakakibara
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  5. Minoru Hasegawa
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  8. Kazunaga Agematsu
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  9. Akihiro Yachie
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Correspondence to Taizo Wada.

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Wada, T., Toga, A., Sakakibara, Y. et al. Clonal expansion of Epstein–Barr virus (EBV)-infected γδ T cells in patients with chronic active EBV disease and hydroa vacciniforme-like eruptions. Int J Hematol 96, 443–449 (2012). https://doi.org/10.1007/s12185-012-1156-0

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  • DOI: https://doi.org/10.1007/s12185-012-1156-0

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