Important
It is possible that the main title of the report X linked Lymphoproliferative Syndromeis not the name you expected.

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
800: (888)663-4637
Askus@marchofdimes.com
http://www.marchofdimes.com

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

National Cancer Institute
6116 Executive Blvd, MSC 8322, Room 3036A
Bethesda
MD
20892-8322
USA
Tel: (301)435-3848
800: (800)422-6237
TDD: (800)332-8615
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
mnugent@hpi.mcw.edu
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
800: (800)546-5268
info@nbmtlink.org
http://www.nbmtlink.org

OncoLink: The University of Pennsylvania Cancer Center Resource
3400 Spruce Street
2 Donner
Philadelphia
PA
19104-4283
USA
Tel: (215)349-5445
Fax: (215)349-5445
editors@oncolink.upenn.edu
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
info@ipopi.org
http://www.ipopi.org/

Jeffrey Modell Foundation
747 Third Ave
34th Floor
New York
NY
10017
USA
Tel: (212)819-0200
Fax: (212)764-4180
800: (866)469-6474
info@jmfworld.org
http://www.info4pi.org

NIH/Hematology Branch, National Heart, Lung and Blood Institute (NHLBI)
10 Center Dr, Building 10-CRC
3-5140, MSC-1202
Bethesda
MD
20892-1202
Tel: (301)402-0764
Fax: (301)402-3088
zamaniw@nhlbi.nih.gov
http://dir.nhlbi.nih.gov/labs/hb/index.asp?

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