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

• Main Authors: Masataka Ishimura (Author), Katsuhide Eguchi (Author), Akira Shiraishi (Author), Motoshi Sonoda (Author), Yoshihiro Azuma (Author), Hiroyuki Yamamoto (Author), Ken-ichi Imadome (Author), Shouichi Ohga (Author)
• Format: Book
• Published: Frontiers Media S.A., 2019-05-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...