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

Overview

Journal Herpesviridae

Publisher Biomed Central

Date 2012 Feb 14

PMID 22325832

Citations 9

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

Affiliations

Soon will be listed here.

Abstract

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.

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