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X linked Lymphoproliferative Syndrome

Abstract

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Synonyms of X linked Lymphoproliferative Syndrome

  • Duncan Disease
  • EBV Susceptibility (EBVS)
  • Epstein-Barr Virus-Induced Lymphoproliferative Disease in Males
  • Immunodeficiency-5 (IMD5)
  • Purtilo Syndrome
  • X-Linked Progressive Combined Variable Immunodeficiency
  • XLP

Disorder Subdivisions

  • No subdivisions found.

General Discussion

X-linked lymphoproliferative (XLP) syndrome is an extremely rare inherited (primary) immunodeficiency disorder characterized by a defective immune system that is powerfully responsive to infection with the Epstein-Barr virus (EBV). This virus is common among the general population and is relatively well-known because it is the cause of infectious mononucleosis (IM), usually with no long-lasting effects. However, in individuals with XLP, exposure to EBV may result in severe, life-threatening fulminant hepatitis; abnormally low levels of antibodies in the blood and body secretions (hypogammaglobulinemia), resulting in increased susceptibility to various infections; malignancies of certain types of lymphoid tissue (B-cell lymphomas); and/or other abnormalities. The range of symptoms and findings associated with XLP may vary considerably from case to case. In addition, the range of effects may change in an affected individual over time. In most cases, individuals with XLP experience an onset of symptoms anytime from ages about 6 months to 10 years of age.

Organizations related to X linked Lymphoproliferative Syndrome

(Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder [e.g., immune deficiency, aplastic anemia, B-cell lymphoma, etc.].)

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