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  • Title: [X-linked lymphoproliferative syndrome, EBV virus infection and defects in cytotoxicity lymphocyte regulation].
    Author: Malbrán A, Belmonte L, Ruibal-Ares B, Baré P, Bracco MM.
    Journal: Medicina (B Aires); 2003; 63(1):70-6. PubMed ID: 12673966.
    Abstract:
    Mutations in SH2D1A, a gene that codifies for the regulatory protein SAP, result in uncontrolled activation of the SLAM (signaling lymphocyte-activation molecule) pathway. This X-linked immunodeficiency becomes evident when the patients are infected with Epstein Barr virus (EBV) and develop a fulminant form of infectious mononucleosis leading to a lymphoproliferative syndrome that is often fatal (X-linked lymphoproliferative syndrome, XLP). In those who survive, hypogammaglobulinemia and oncohematologic diseases are frequently observed. In this revision, the immuno-regulatory mechanisms involved in XLP immunopathology and the role of different effector cells (CD8 T lymphocytes, NK cells) are discussed.
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