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首页> 外文期刊>International Journal of Hematology >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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Clonal expansion of Epstein–Barr virus (EBV)-infected γδ T cells in patients with chronic active EBV disease and hydroa vacciniforme-like eruptions

机译：慢性活动性EBV病和水痘疫苗样爆发的患者中爱泼斯坦-巴尔病毒（EBV）感染的γδT细胞的克隆扩增

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摘要

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.
机译：慢性活动性爱泼斯坦-巴尔病毒（EBV）病（CAEBV）是一种以发烧，淋巴结病和脾肿大为特征的全身性EBV阳性淋巴增生性疾病。 CAEBV患者可能会出现皮肤症状，包括对蚊虫叮咬和水痘疫苗样（HV）样喷发过敏。 HV是一种罕见的光敏性皮肤病，其特征是暴露于阳光后会出现囊泡和结皮，并在儿童时期发作，并与潜在的EBV感染相关。尽管最近已证明γδT细胞是HV中主要的EBV感染细胞群，但CAEBV中EB感染的γδT细胞具有类似HV爆发或HV的免疫表型特征仍未确定。我们描述了三名CAEBV患者，他们的γδT细胞被发现是EBV的主要细胞靶标。在其中两名患者中观察到类似HV的爆发。在所有患者中均证实了高度激活的γδT细胞的克隆扩展亚群以及T细胞受体Vγ9和Vδ2阳性细胞。我们还显示，在一名可从皮肤活检标本获得的患者中，无性扩张的γδT细胞渗透入HV样爆发。这些结果表明，由EBV感染的克隆扩增的γδT细胞在CAEBV和HV样爆发患者中的致病作用。

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  • 来源
    《International Journal of Hematology》 |2012年第4期|p.443-449|共7页
  • 作者

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

  • 作者单位

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

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

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

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

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

    Department of Dermatology, School of Medicine, Institute of Medical, Pharmaceu;

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  • 正文语种 eng
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  • 关键词

    Epstein–Barr virus; Clonal proliferation; γδ T cells; Hydroa vacciniforme;

    机译：EB病毒;克隆增殖;γδT细胞;牛痘菌;

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