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. Normally, after initial infection, EBV remains in certain immune system cells (lymphocytes) called B cells. 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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