Medical Research And Gene Detection In A Family With X-linked Liphoproliferative Disease

X-linked lymphoproliferative disease (XLP), also named Duncan's syndrome, is a rare, often fatal, inherited immunodeficiency in which affected boys show abnormal responses to Epstein-Barr virus infection. Usually female is carrier of the mutation gene, and male is the patient. The disease is cured without good methods now and the result is bad.The Epstein-Barr virus (EBV) carrier state is almost general in humans. Infection occurs usually in adolescents and is followed by persistence of the virus through lifetime without appreciable deleterious effects . The virus carrier state of the individuals can be easily detected by assessing the presence of EBV specific antibodies and reactive lymphocytes directed against viral structural proteins and against virally encoded proteins expressed in the infected cells. The virus can transform B lymphocytes in vitro for unlimited proliferation. In patients with a normal immune response, immunological mechanisms, particularly innate and adaptive cellular immunity parameters ensure that this effect is not manifested in vivo. But for the XLP patients, mutation gene causes the patients' immune system under its physiological function, and it can't constrain EB virus proliferation, then the clinical manifestation and prognosis are both serious bad. Studies of the immune responses in XLP patients suggest that abnormal T and B cell proliferation occurs in response to EBV-induced lymphoblasts, This impressive polyclonal T cell and B cell...