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Background  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.    Methods  EBV-infected cells in two patients (XLP-1 and XLP-2) presenting EBV-HLH were evaluated by in EBER-1  in situ  hybridization or quantitative PCR methods.    Results  Both XLP patients showed that the dominant population of EBV-infected cells was CD19 +  B cells, whereas EBV-infected CD8 +  T cells were very few.    Conclusions  In XLP-related EBV-HLH, EBV-infected cells appear to be predominantly B cells. B cell directed therapy such as rituximab may be a valuable option in the treatment of EBV-HLH in XLP patients.

herpesviridae

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