Systemic Epstein–Barr Virus-Positive T/NK Lymphoproliferative Diseases With SH2D1A/XIAP Hypomorphic Gene Variants

X-linked lymphoproliferative disease (XLP) is one of the X-linked primary immunodeficiency diseases (PIDs) with defective immune response to Epstein–Barr virus (EBV) infection. Chronic active EBV infection (CAEBV) and EBV-hemophagocytic lymphohistiocytosis (HLH) are recognized as systemic EBV-positi...

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Bibliographic Details

Published in:	Frontiers in Pediatrics
Main Authors:	Masataka Ishimura, Katsuhide Eguchi, Akira Shiraishi, Motoshi Sonoda, Yoshihiro Azuma, Hiroyuki Yamamoto, Ken-ichi Imadome, Shouichi Ohga
Format:	Article
Language:	English
Published:	Frontiers Media S.A. 2019-05-01
Subjects:	Epstein–Barr virus chronic active EBV infection lymphoproliferative disease hemophagocytic lymphohistiocytosis SAP XIAP
Online Access:	https://www.frontiersin.org/article/10.3389/fped.2019.00183/full