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X linked Lymphoproliferative Syndrome

National Organization for Rare Disorders, Inc.

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)997-4488

Fax: (914)997-4763

Tel: (888)663-4637

Email: Askus@marchofdimes.com

Internet: http://www.marchofdimes.com



Immune Deficiency Foundation

40 W. Chesapeake Avenue

Suite 308

Towson, MD 21204

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

Office of Communications and Government Relations

6610 Rockledge Drive, MSC 6612

Bethesda, MD 20892-6612

Tel: (301)496-5717

Fax: (301)402-3573

Tel: (866)284-4107

TDD: (800)877-8339

Email: ocpostoffice@niaid.nih.gov

Internet: http://www.niaid.nih.gov/



National Cancer Institute

6116 Executive Blvd Suite 300

Bethesda, MD 20892-8322

USA

Tel: (301)435-3848

Tel: (800)422-6237

TDD: (800)332-8615

Email: cancergovstaff@mail.nih.gov

Internet: http://www.cancer.gov



Center for International Blood and Marrow Transplant Research

Froedtert and the Medical College of Wisconsin Clinical Cancer Center

9200 W. Wisconsin Avenue

Milwaukee, WI 53226

Tel: (414)805-0700

Fax: (414)805-0714

Email: contactus@cibmtr.org

Internet: 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: (215)349-8895

Fax: (215)349-5445

Email: hampshire@uphs.upenn.edu

Internet: http://www.oncolink.upenn.edu



International Patient Organization for Primary Immunodeficiencies

Firside Main Road

Downderry

Cornwall, PL11 3LE

United Kingdom

Tel: 441503250668

Fax: 441503250961

Email: info@ipopi.org

Internet: http://www.ipopi.org/



Jeffrey Modell Foundation

780 Third Avenue

New York, NY 10017

USA

Tel: (212)819-0200

Fax: (212)764-4180

Tel: (866)469-6474

Email: info@jmfworld.org

Internet: http://www.info4pi.org



NIH/National Heart, Lung and Blood Institute ~ Hematology Branch

10 Center Dr, Building 10-CRC

3-5140, MSC-1202

Bethesda, MD 20892-1202

Tel: (301)496-5093

Fax: (301)496-8396

Tel: (800)644-2337

Email: YoungNS@mail.nih.gov

Internet: http://dir.nhlbi.nih.gov/labs/hb/index.asp?



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



XLP Research Trust

60 Winchester Road

Romsey

Hampshire, SO51 8JA

United Kingdom

Tel: 4401794521077

Email: info@xlpresearchtrust.org

Internet: http://www.xlpresearchtrust.org



European Society for Immunodeficiencies

1-3 rue de Chantepoulet

Geneva, CH 1211

Switzerland

Tel: 410229080484

Fax: 41229069140

Email: esid@kenes.com

Internet: http://www.esid.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  9/23/2007

Copyright  1989, 1995, 1997, 1998, 1999, 2006, 2007 National Organization for Rare Disorders, Inc.

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