作者
Kei Sato, Naoko Misawa, Chuanyi Nie, Yorifumi Satou, Dai Iwakiri, Masao Matsuoka, Rei Takahashi, Kiyotaka Kuzushima, Mamoru Ito, Kenzo Takada, Yoshio Koyanagi
发表日期
2011/5/26
期刊
Blood, The Journal of the American Society of Hematology
卷号
117
期号
21
页码范围
5663-5673
出版商
American Society of Hematology
简介
EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a rare yet devastating disorder caused by EBV infection in humans. However, the mechanism of this disease has yet to be elucidated because of a lack of appropriate animal models. Here, we used a human CD34+ cell-transplanted humanized mouse model and reproduced pathologic conditions resembling EBV-HLH in humans. By 10 weeks postinfection, two-thirds of the infected mice died after exhibiting high and persistent viremia, leukocytosis, IFN-γ cytokinenemia, normocytic anemia, and thrombocytopenia. EBV-infected mice also showed systemic organ infiltration by activated CD8+ T cells and prominent hemophagocytosis in BM, spleen, and liver. Notably, the level of EBV load in plasma correlated directly with both the activation frequency of CD8+ T cells and the level of IFN-γ in plasma. Moreover, high levels of EBV-encoded small …
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K Sato, N Misawa, C Nie, Y Satou, D Iwakiri… - Blood, The Journal of the American Society of …, 2011