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

21 May 2019

Abstract excerpt

X-linked lymphoproliferative disease (XLP) is one of the X-linked primary immunodeficiency diseases (PIDs) reportedly to have a defective immune response to Epstein-Barr virus (EBV) infection. Mutations in SH2D1A and XIAP genes cause XLP. Systemic EBV-positive T-cell and natural killer (NK)-cell lymphoproliferative diseases (LPD) consist of the 3 major types; EBV-positive hemophagocytic lymphohistiocytosis (HLH),...

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PMID: 31231620
DOI: 10.3389/fped.2019.00183

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