Virological and immunological characteristics of a 19-year-old Japanese female with fatal outcome with Epstein-Barr virus-associated hemophagocytic syndrome
Virological and immunological characteristics of a 19-year-old Japanese female with fatal outcome with Epstein-Barr virus-associated hemophagocytic syndrome
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DOI：
10.1016/j.jcv.2004.06.004
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发表时间：
2004-11-01
影响因子：
8.8
通讯作者：
Hoshika, A
中科院分区：
文献类型：
--
作者：
Sato, S;Kawashima, H;Hoshika, A
噬血细胞综合征 (HPS) 是由 T 细胞和巨噬细胞过度激活引起的。与这种疾病相关的临床特征是由于细胞因子的过量产生所致，包括干扰素-γ (INF-γ)、肿瘤坏死因子-α (TNF-α)、白细胞介素-6 (IL-6)。 HPS 表现为发热、肝功能障碍、凝血异常、全血细胞减少和良性组织细胞增殖，并伴有骨髓、淋巴结、脾脏和肝脏中显着的噬血细胞增多。我们描述了一名患有致命 HPS 的 19 岁女性。她因患有系统性红斑狼疮（SLE）而每隔一天接受一次皮质类固醇治疗，但没有嘴唇红肿。致病基础疾病是急性原发性 Epstein-Barr 病毒 (EBV) 感染。通过聚合酶链反应（PCR）检测EBV基因组。为了测量病毒载量，我们使用实时 PCR 检测来量化尸检时外周血淋巴细胞、肺、肾、脑和肝脏中 EBV DNA 的数量。进一步的原位杂交（ISH）和免疫组织化学研究表明，在骨髓、肺、肾、脑、肝和脾的CD8（+）T细胞中检测到Epstein-Barr病毒编码的小RNA（EBER）。在每个器官中，与β-肌动蛋白mRNA水平相比，炎症细胞因子（INF-γ、TNF-α、IL-6）的mRNA水平被高度检测到。这些结果表明，每个器官（外周血淋巴细胞、肺、肾、脑和肝）中 EBV 感染的 CD8+ T 细胞可能在 HPS 的病理生理学中发挥着不可或缺的作用。 (C) 2004 Elsevier B.V. 保留所有权利。
Hemophagocytic syndrome (HPS) is caused by the hyperactivation of T-cells and macrophages. The clinical characteristics associated with this disease result from overproduction of cytokines including interferon-gamma (INF-gamma), tumor necrosis factor-alpha (TNF-alpha), interleukin-6 (IL-6). HPS presents with fever, liver dysfunction, coagulation abnormalities, pancytopenia, and a benign histiocytic proliferation with prominent hemophagocytosis in bone marrow, lymph node, spleen, and liver. We describe a 19-year-old female with fatal HPS. She had been given corticosteroid every other day for systemic lupus erythematosus (SLE) without flare Lip. The causative underlying disease was acute primary Epstein-Barr virus (EBV) infection. EBV genomes were detected by the polymerase chain reaction (PCR). To measure the virus load we use a real-time PCR assay to quantify the amount of EBV DNA in peripheral blood lymphocytes, lung, kidney, brain and liver at autopsy. Further in situ hybridisation (ISH) and immunohistochemical studies demonstrated that Epstein-Barr virus encoded small RNA (EBER) was detected in CD8(+) T-cells in bone marrow, lung, kidney, brain, liver and spleen. In each organ, mRNA levels of inflammatory cytokines (INF-gamma, TNF-alpha, IL-6) were highly detected compared with beta-Actin mRNA levels. These results Suggest that EBV-infected CD8(+) T-cells in each organ (peripheral blood lymphocytes, lung, kidney, brain and liver) may have an integral role in the pathophysiology of the HPS. (C) 2004 Elsevier B.V. All rights reserved.