Diseases & Conditions


X linked Lymphoproliferative Syndrome

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

    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
    • Center for International Blood and Marrow Transplant Research
      Medical College of Wisconsin
      Milwaukee WI 53226
      Phone #: 414-456-8325
      800 #: --
      e-mail: mnugent@hpi.mcw.edu
      Home page: http://www.ibmtr.org;
    • European Society for Immunodeficiencies (ESID)
      c/o Dr. Esther de Vries
      Hertogenbosch None 5200 ME's
      Phone #: +31- 73--6992965
      800 #: N/A
      e-mail: info@esid.org
      Home page: http://www.esid.org
    • Immune Deficiency Foundation
      40 West Chesapeake Avenue
      Towson MD 21204
      Phone #: 410-321-6647
      800 #: 800-296-4433
      e-mail: idf@primaryimmune.org
      Home page: http://www.primaryimmune.org
    • International Patient Organization for Primary Immunodeficiencies
      Cornwall None PL11 3LE
      Phone #: 44 -150-3 250 668
      800 #: --
      e-mail: info@ipopi.org
      Home page: http://www.ipopi.org/
    • Jeffrey Modell Foundation
      747 Third Ave
      New York NY 10017
      Phone #: 212-819-0200
      800 #: 866-469-6474
      e-mail: info@jmfworld.org
      Home page: http://www.info4pi.org
    • March of Dimes Birth Defects Foundation
      1275 Mamaroneck Avenue
      White Plains NY 10605
      Phone #: 914-428-7100
      800 #: 888-663-4637
      e-mail: Askus@marchofdimes.com
      Home page: http://www.marchofdimes.com
    • NIH/Hematology Branch, National Heart, Lung and Blood Institute (NHLBI)
      10 Center Dr, Building 10-CRC
      Bethesda MD 20892-1202
      Phone #: 301-402-0764
      800 #: --
      e-mail: zamaniw@nhlbi.nih.gov
      Home page: http://dir.nhlbi.nih.gov/labs/hb/index.asp?
    • NIH/National Institute of Allergy and Infectious Diseases
      6610 Rockledge Drive
      Bethesda MD 20892-6612
      Phone #: 301-496-5717
      800 #: --
      e-mail: N/A
      Home page: http://www.niaid.nih.gov/
    • National Bone Marrow Transplant Link
      20411 W. 12 Mile Rd
      Southfield MI 48076
      Phone #: 248-358-1886
      800 #: 800-546-5268
      e-mail: info@nbmtlink.org
      Home page: http://www.nbmtlink.org
    • National Cancer Institute
      6116 Executive Blvd, MSC 8322, Room 3036A
      Bethesda MD 20892-8322
      Phone #: 301-435-3848
      800 #: 800-422-6237
      e-mail: N/A
      Home page: http://www.cancer.gov
    • OncoLink: The University of Pennsylvania Cancer Center Resource
      3400 Spruce Street
      Philadelphia PA 19104-4283
      Phone #: 215-349-5445
      800 #: --
      e-mail: editors@oncolink.upenn.edu
      Home page: http://www.oncolink.upenn.edu
    • XLP Research Trust
      60 Winchester Road
      Hampshire None 5051 8JA
      Phone #: +44- 17-94 521077
      800 #: N/A
      e-mail: info@xlpresearchtrust.org
      Home page: http://www.xlpresearchtrust.org

    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html