The x-linked lymphoproliferative disease describes a hereditary immunodeficiency. In most cases, it manifests itself as an infection with the Epstein-Barr virus (EBV), to which the immune system does not respond. For example, EBV infections cause these symptoms:
Fulminant infectious mononucleosis, macrophage activation syndrome, or hemophagocytic lymphohistiocytosis, progressive hypogammaglobulinemia, and / or lymphoma.
Haemophagocytic lymphohistiocytosis is triggered by the activation and excessive proliferation of T cells and macrophages and is potentially fatal.
Fever, hepatosplenomegaly and lymphadenopathy may result in clotting disorders, central nervous system function and multiple organ failure. Less common are aplastic anemia and lymphocytic vasculitis.
Inheritance is X-linked recessive and in most cases (60%) is a mutation of the SH2D1A gene, which is responsible for the activation of T lymphocytes.
The x-linked lymphoproliferative disease describes a hereditary immunodeficiency. In most cases, it manifests itself as an infection with the Epstein-Barr virus (EBV), to which the immune system does not respond. For example, EBV infections cause these symptoms:Fulminant infectious mononucleosis, macrophage activation syndrome, or hemophagozyme lymphohistiocytosis, progressive hypogammaglobulinemia and / or lymphomas. Hemophagocytic lymphohistiocytosis is triggered by the activation and excessive proliferation of T cells and macrophages and is potentially fatal.
Fever, hepatosplenomegaly, and lymphadenopathy may result in clotting disorders, central nervous function, and multiple organ dysfunction. Less common are aplastic anemia and lymphocytic vasculitis.
Inheritance is x-linked recessive and in most cases (60%) is a mutation of the SH2D1A gene that is responsible for the activation of T lymphocytes. & nbsp;