Immunophenotypic analysis of Epstein–Barr virus (EBV)‐infected CD8+ T cells in a patient with EBV‐associated hemophagocytic lymphohistiocytosis
Immunophenotypic analysis of Epstein–Barr virus (EBV)‐infected CD8+ T cells in a patient with EBV‐associated hemophagocytic lymphohistiocytosis
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EB 病毒相关噬血细胞性淋巴组织细胞增多症患者感染 EB 病毒 (EBV) 的 CD8+ T 细胞的免疫表型分析
DOI：
10.1111/j.1600-0609.2007.00868.x
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发表时间：
2007
影响因子：
3.1
通讯作者：
A. Yachie
中科院分区：
文献类型：
--
作者：
T. Wada;T. Kurokawa;T. Toma;F. Shibata;Y. Tone;Y. Hashida;H. Kaya;Takashi Yoshida;A. Yachie
噬血细胞性淋巴组织细胞增生症（HLH）是一种严重且通常致命的疾病，其特征是T细胞和巨噬细胞的不受控制的活化。在EB病毒（EBV）相关HLH（EBV-HLH）中，已描述了T细胞群中异位EBV感染和EBV感染T细胞克隆增殖的致病作用。然而，EBV感染的T细胞的免疫表型尚未完全表征。在这里，我们描述了一个EBV-HLH的情况下，提出了大量的克隆性增殖的CD 8 + T细胞与TCR VB 14。EBV编码的小RNA 1原位杂交分析显示，该患者中仅CD 8 + T细胞携带EBV。EBV感染的TCR VB 14 + CD 8 + T细胞表现出独特的免疫表型特征，包括缺乏CD 5表达和明显明亮的HLA-DR表达。在开始用泼尼松龙、依托泊苷和环孢菌素A治疗后，感染细胞的百分比与其他血清标志物如铁蛋白平行地进行性下降。这些发现表明，具有特异性TCR VB的CD 8 + T细胞上缺乏CD 5表达可能是EBV-HLH中T细胞活化和增殖失调的有用标志物。
Hemophagocytic lymphohistiocytosis (HLH) is a severe and often fatal condition characterized by uncontrolled activation of T cells and macrophages. In Epstein–Barr virus (EBV)‐associated HLH (EBV‐HLH), the pathogenic roles of ectopic EBV infection in the T‐cell population and of clonal proliferation of EBV‐infected T cells has been described. However, the immunophenotype of EBV‐infected T cells has not been fully characterized. Here we describe a case of EBV‐HLH presenting with a massive clonal proliferation of CD8+ T cells with TCR VB14. Analysis of in situ hybridization for EBV‐encoded small RNA1 showed that only CD8+ T cells harbored EBV in this patient. The EBV‐infected TCR VB14+ CD8+ T cells exhibited unique immunophenotypic features including lacked CD5 expression and a markedly bright expression of HLA‐DR. After initiation of treatment with prednisolone, etoposide, and cyclosporin A, the percentage of infected cells declined progressively in parallel with other serum markers such as ferritin. These findings suggest that lacking expression of CD5 on CD8+ T cells with specific TCR VB may serve as a useful marker of dysregulated T‐cell activation and proliferation in EBV‐HLH.