Title |
Characterization of Epstein-Barr virus (EBV)-infected cells in EBV-associated hemophagocytic lymphohistiocytosis in two patients with X-linked lymphoproliferative syndrome type 1 and type 2
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Published in |
Herpesviridae, February 2012
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DOI | 10.1186/2042-4280-3-1 |
Pubmed ID | |
Authors |
Xi Yang, Taizo Wada, Ken-Ichi Imadome, Naonori Nishida, Takeo Mukai, Mitsuhiro Fujiwara, Haruka Kawashima, Fumiyo Kato, Shigeyoshi Fujiwara, Akihiro Yachie, Xiaodong Zhao, Toshio Miyawaki, Hirokazu Kanegane |
Abstract |
X-linked lymphoproliferative syndrome (XLP) is a rare inherited immunodeficiency by an extreme vulnerability to Epstein-Barr virus (EBV) infection, frequently resulting in hemophagocytic lymphohistiocytosis (HLH). XLP are now divided into type 1 (XLP-1) and type 2 (XLP-2), which are caused by mutations of SH2D1A/SLAM-associated protein (SAP) and X-linked inhibitor of apoptosis protein (XIAP) genes, respectively. The diagnosis of XLP in individuals with EBV-associated HLH (EBV-HLH) is generally difficult because they show basically similar symptoms to sporadic EBV-HLH. Although EBV-infected cells in sporadic EBV-HLH are known to be mainly in CD8+ T cells, the cell-type of EBV-infected cells in EBV-HLH seen in XLP patients remains undetermined. |
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