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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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Principais autores: Masataka Ishimura, Katsuhide Eguchi, Akira Shiraishi, Motoshi Sonoda, Yoshihiro Azuma, Hiroyuki Yamamoto, Ken-ichi Imadome, Shouichi Ohga
Formato: Artigo
Idioma:Inglês
Publicado em: Frontiers Media S.A. 2019-05-01
coleção:Frontiers in Pediatrics
Assuntos:
Epstein–Barr virus
chronic active EBV infection
lymphoproliferative disease
hemophagocytic lymphohistiocytosis
SAP
XIAP
Acesso em linha:https://www.frontiersin.org/article/10.3389/fped.2019.00183/full
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