Important
It is possible that the main title of the report X linked Lymphoproliferative Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

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

Disorder Subdivisions

• None

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.

Resources

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)428-7100
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com

Immune Deficiency Foundation
40 West Chesapeake Avenue
Suite 308
Towson, MD 21230
Tel: (410)321-6647
Fax: (410)321-9165
Tel: (800)296-4433
Email: idf@primaryimmune.org
Internet: http://www.primaryimmune.org

NIH/National Institute of Allergy and Infectious Diseases
6610 Rockledge Drive
MSC 6612
Bethesda, MD 20892-6612
Tel: (301)496-5717
Fax: (301)402-3573
TDD: (800)877-8339
Internet: http://www.niaid.nih.gov/

National Cancer Institute
6116 Executive Blvd, MSC 8322, Room 3036A
Bethesda, MD 20892-8322
USA
Tel: 3014353848
Tel: 8004226237
TDD: 8003328615
Internet: http://www.cancer.gov

Center for International Blood and Marrow Transplant Research
Medical College of Wisconsin
8701 Watertown Plank Road
Milwaukee, WI 53226
Tel: (414)456-8325
Fax: (414)456-6530
Email: mnugent@hpi.mcw.edu
Internet: http://www.ibmtr.org; http://www.cibmtr.org

National Bone Marrow Transplant Link
20411 W. 12 Mile Rd
Suite 108
Southfield, MI 48076
Tel: (248)358-1886
Fax: (248)358-1889
Tel: (800)546-5268
Email: info@nbmtlink.org
Internet: http://www.nbmtlink.org

OncoLink: The University of Pennsylvania Cancer Center Resource
3400 Spruce Street
2 Donner
Philadelphia, PA 19104-4283
USA
Tel: 2153495445
Fax: 2153495445
Email: editors@oncolink.upenn.edu
Internet: http://www.oncolink.upenn.edu

International Patient Organization for Primary Immunodeficiencies
Firside
Main Road
Downderry
Cornwall, PL11 3LE
United Kingdom
Tel: 44 1503 250 668
Fax: 44 1503 250 668
Email: info@ipopi.org
Internet: http://www.ipopi.org/

Jeffrey Modell Foundation
747 Third Ave
34th Floor
New York, NY 10017
USA
Tel: 2128190200
Fax: 2127644180
Tel: 8664696474
Email: info@jmfworld.org
Internet: http://www.info4pi.org

NIH/Hematology Branch, National Heart, Lung and Blood Institute (NHLBI)

Tel: (301)402-0764
Fax: (301)402-3088
Email: zamaniw@nhlbi.nih.gov
Internet: http://www.nhlbi.nih.gov/nhlbi/seekpat/hematol.htm

XLP Research Trust
60 Winchester Road
Romsey
Hampshire, 5051 8JA
United Kingdom
Tel: +44 1794 521077
Email: info@xlpresearchtrust.org
Internet: http://www.xlpresearchtrust.org

European Society for Immunodeficiencies (ESID)
c/o Dr. Esther de Vries
Jeroen Bosch Hospital
Dept. Paediatrics
P.O. Box 90153
Hertogenbosch, 5200 ME's
Netherlands
Tel: +31 73-6992965
Fax: +31 73-6992948
Email: info@esid.org
Internet: http://www.esid.org