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XLP can be divided into two types based on its genetic cause and pattern of signs and symptoms: XLP1 (also known as classic XLP) and XLP2. Death usually results from hemophagocytic lymphohistiocytosis.
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Without treatment, most people with XLP survive only into childhood. Individuals with dysgammaglobulinemia are prone to recurrent infections.Ĭancers of immune system cells ( lymphomas ) occur in about one-third of people with XLP. Antibodies (also known as immunoglobulins) are proteins that attach to specific foreign particles and germs, marking them for destruction. In some individuals with XLP, hemophagocytic lymphohistiocytosis or related symptoms may occur without EBV infection.Ībout one-third of people with XLP experience dysgammaglobulinemia, which means they have abnormal levels of some types of antibodies. The spleen, heart, kidneys, and other organs and tissues may also be affected. Hemophagocytic lymphohistiocytosis causes fever, destroys blood-producing cells in the bone marrow, and damages the liver. This proliferation of immune cells often causes a life-threatening reaction called hemophagocytic lymphohistiocytosis. People with XLP may respond to EBV infection by producing abnormally large numbers of T cells, B cells, and other lymphocytes called macrophages. However, the virus is generally inactive (latent) because it is controlled by other lymphocytes called T cells that specifically target EBV-infected B cells.
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Normally, after initial infection, EBV remains in certain immune system cells (lymphocytes) called B cells.
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In some people it causes infectious mononucleosis (commonly known as "mono"). EBV is a very common virus that eventually infects most humans. More than half of individuals with this disorder experience an exaggerated immune response to the Epstein-Barr virus (EBV). X-linked lymphoproliferative disease (XLP) is a disorder of the immune system and blood-forming cells that is found almost exclusively in males.
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