PDF(Pubmed)
Abstract：
Hydroa vacciniforme lymphoproliferative disorder (HV-LPD) is a cutaneous variant of chronic active Epstein-Barr virus disease. We examined the coexpression of T- and natural killer (NK)-cell antigens in five patients with classic HV (cHV) and five with systemic HV (sHV). T-cell receptor (TCR) repertoire analysis was performed with high‑throughput sequencing. All five cHV patients had increased γδT cells (> 5%), whereas five sHV patients showed γδT- and αβT-cell dominance in two patients each, and a mixture of abnormal γδT and αβT cells in one. Circulating CD3 + T cells expressed CD16/CD56 at 7.8-42.3% and 1.1-9.7% in sHV and cHV, respectively. The percentage of CD16/CD56 + T cells was higher in the large granular lymphocyte or atypical T-cell fractions in sHV, but no TCR Vα24 invariant chain characteristic of NKT cells was detected. Considerable numbers of CD3 + cells expressing CD56 were observed in sHV skin infiltrates. Of the circulating γδT cells tested, TCR Vδ1 + cells characteristic of the epithelial type of γδT cells were dominant in two sHV cases. Thus, atypical αβT and γδT cells in HV-LPD can express NK-cell antigens, such as CD16 and CD56, and Vδ1 + epithelial-type γδT cells are a major cell type in some HV-LPD cases.
摘要：
水生疫苗淋巴增生性疾病（HV-LPD）是慢性活动性爱泼斯坦-巴尔病毒病的皮肤变体。我们检查了5例经典HV（cHV）和5例全身性HV（sHV）患者中T和自然杀伤（NK）细胞抗原的共表达。使用高通量测序进行T细胞受体（TCR）库分析。所有5例cHV患者的γδT细胞增加（>5％），而5例sHV患者分别在2例患者中表现出γδT和αβT细胞优势，以及异常γδT和αβT细胞的混合物。在sHV和cHV中，循环CD3+T细胞表达CD16/CD56的比例分别为7.8-42.3％和1.1-9.7％，分别。在sHV中，大颗粒淋巴细胞或非典型T细胞组分中CD16/CD56+T细胞的百分比较高，但未检测到NKT细胞的TCRVα24不变链特征。在sHV皮肤浸润物中观察到相当数量的表达CD56的CD3+细胞。在测试的循环γδT细胞中，在两个sHV病例中，以上皮型γδT细胞为特征的TCRVδ1细胞占优势。因此，HV-LPD中的非典型αβT和γδT细胞可以表达NK细胞抗原，在某些HV-LPD病例中，CD16和CD56以及Vδ1上皮型γδT细胞是主要的细胞类型。
