X-linked lymphoproliferative disease (Q84629)

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X-linked lymphoproliferative disease is a hereditary immunodeficiency characterised, in the majority of cases, by an inadequate immune response to infection with the Epstein-Barr virus (EBV). Infection with the Epstein-Barr virus can result in one or several of the following manifestations: fulminant infectious mononucleosis, macrophage-activation syndrome or hemophagocytic lymphohistiocytosis (HLH; see this term), and/or progressive hypogammaglobulinemia and/or lymphomas.
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    X-linked lymphoproliferative disease
    X-linked lymphoproliferative disease is a hereditary immunodeficiency characterised, in the majority of cases, by an inadequate immune response to infection with the Epstein-Barr virus (EBV). Infection with the Epstein-Barr virus can result in one or several of the following manifestations: fulminant infectious mononucleosis, macrophage-activation syndrome or hemophagocytic lymphohistiocytosis (HLH; see this term), and/or progressive hypogammaglobulinemia and/or lymphomas.

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