| Budget Amount *help |
¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2011: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2010: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2009: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
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| Research Abstract |
EBV-HLH is characterized by extreme cytokinemia and cytopenia, frequently leading to aggressive deterioration and fatal outcome. It is of paramount importance to make correct diagnosis and start therapeutic intervention as soon as possible, as the delay in the treatment may result in irreversible damage to vital organs within several hours. We now understand that the essential pathogenesis of EBV-HLH is selective activation and expansion of an ectopically EBV-infected CD8^+T cell clone. Thus the definitive diagnosis of EBV-HLH can be made by identifying the target of EBV infection and by proving the clonality of the EBV-infected cells.
In this study, we analyzed the profiles of surface antigen expression of the infected CD8^+T cells and serum cytokines. With these results, we tried to establish a simplified method to establish parameters for the early diagnosis of EBV-HLH. As was expected, EBV infection in most cases of EBV-HLH was confined to a single clone of CD8+T cells, expressing a particular TCR Vβ. CDR3 size distribution analysis indicates that these cells are of a single clone. The abnormal clones exhibited high levels of HLA-DR and decreased levels of CD5. Therefore, EBV-infected cells were identifiable as HLA-DR^++CD5^-cell population within CD8^+T cells. Further analysis revealed that similar cell population was observed in patients with FHL2 during acute viral infection, including EBV.
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