Su I J, Chen R L, Lin D T, Lin K S, Chen C C
Department of Pathology, National Taiwan University Hospital and College of Medicine, Taipei.
Am J Pathol. 1994 Jun;144(6):1219-25.
We have reported the prevalence of a fulminant hemophagocytic syndrome (HS) in previously healthy young children in Taiwan, most of which probably represent a lethal form of primary or active Epstein-Barr virus (EBV) infection. To further confirm their EBV association, in situ EBV hybridization (ISH) was performed on tissue biopsies from 15 pediatric HS patients (median age, 3 years and 4 months) using digoxigenin-labeled RNA probes EBER1. Double labeling immunostaining and ISH was then performed to define the immunophenotype of the lymphoid cells containing the EBV transcripts. Among the 13 patients who had serological evidence of acute or active EBV infection, 9 had demonstrable EBER1 transcripts in bone marrow, liver, and/or skin biopsies. EBER1-specific signal was not detectable in the two specimens from EBV-seronegative patients. The distribution of EBV-containing cells could be extensive or scattered. To our surprise, the EBER1 transcripts existed exclusively in T lymphoid cells in all nine cases examined rather than in B cells as previously believed in infectious mononucleosis. Considering the young affected age of the HS patients and the serological response to EBV, we suggest that EBV can infect T cells in primary EBV infection and the proliferation of these EBV-infected T cells may be responsible for the ominous outcome in childhood HS patients in Taiwan.
我们已经报道了台湾地区既往健康的幼儿中暴发性噬血细胞综合征（HS）的患病率，其中大多数可能代表原发性或活动性EB病毒（EBV）感染的致死形式。为了进一步证实其与EBV的关联，我们使用地高辛标记的RNA探针EBER1对15例儿科HS患者（中位年龄为3岁4个月）的组织活检标本进行了原位EBV杂交（ISH）。然后进行双重标记免疫染色和ISH以确定含有EBV转录本的淋巴细胞的免疫表型。在13例有急性或活动性EBV感染血清学证据的患者中，9例在骨髓、肝脏和/或皮肤活检标本中检测到可证实的EBER1转录本。在来自EBV血清阴性患者的两份标本中未检测到EBER1特异性信号。含EBV细胞的分布可能广泛或分散。令我们惊讶的是，在所检查的所有9例病例中，EBER1转录本仅存在于T淋巴细胞中，而不是像既往认为的传染性单核细胞增多症那样存在于B细胞中。考虑到HS患者的发病年龄较轻以及对EBV的血清学反应，我们认为EBV在原发性EBV感染中可感染T细胞，这些EBV感染的T细胞的增殖可能是台湾地区儿童HS患者不良预后的原因。
