Agammaglobulinemias, Primary

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It is possible that the main title of the report Agammaglobulinemias, Primary 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

Antibody Deficiency
Gammaglobulin Deficiency
Immunoglobulin Deficiency

Disorder Subdivisions

Acquired Immunodeficiency Syndrome (AIDS)
Hypogammaglobulinemia
Late-Onset Immunoglobulin Deficiency
Idiopathic Immunoglobulin Deficiency
Bruton's Agammaglobulinemia Common
Duncan's Disease
Secretory IgA Deficiency
Anti-Epstein-Barr Virus Nuclear Antigen (EBNA) Antibody Deficiency
Adenosine Deaminase Deficiency, Severe Combined Immunodeficiency with
Dysgammaglobulinemia, Janeway
Immunodeficiency Common, Variable, Unclassifiable
Hypogammaglobulinemia Transient of Infancy
IgA Deficiency, Selective
IgG Subclass, Selective Deficiency of
X-Linked Recessive Severe Combined Immunodeficiency
Hyper IgM Syndrome
Janeway I
Antibody Deficiency with near normal Immunoglobulins
IgM Deficiency, Selective
Kappa Light Chain Deficiency
Secretory Component Deficiency
Variable Onset Immunoglobulin Deficiency Dysgammaglobulinemia
Antibody Deficiency with Normal Immunoglobulins, Specific
Thymic Hypoplasia, Agammaglobulinemias, Primary Included
DiGeorge Syndrome
Chronic Mucocutaneous Candidiasis
Cellelar Immunodeficiency with Purine Nucleoside Phosphorylase Deficiency
X-Linked Infantile Agammaglobulinemia
X-Linked Agammaglobulinemia with Growth Hormone Deficiency
Immunodeficiency with Hyper-IgM
X-Linked Agammaglobulinemia
Lymphoproliferate X-Linked Syndrome
Severe Combined Immunodeficiency (SCID)
Autosomal Recessive Agammaglobulinemia
IgG Subclasses, Selective Deficiency of

General Discussion

The primary agammaglobulinemias refer to a group of three inherited immune deficiencies characterized by a low concentration of antibodies in the blood due to the lack of particular lymphocytes in the blood and lymph. Antibodies are proteins (immunoglobulins, [IgM], [IgG] etc) that are critical and key components of the immune system. They are essential if the immune system is to do its job of fighting off bacteria, viruses, and other foreign substances that threaten the body. The specialized precursor cells that produce gammaglobulins, fail to function properly leading to the deficiency in the number of mature lymphocyte cells. In addition the specialized white blood cells that are the body’s primary defense weapons (B-lymphocytes and T-lymphocytes) fail to function properly.

The three types of primary agammaglobulinemias are: X-linked agammaglobulinemia (XLA), plus the much rarer X-linked agammaglobulinemia with growth hormone deficiency (about 10 cases reported), and autosomal recessive agammaglobulinemia (about 5-6 cases reported) (ARAG). All of these disorders are characterized by a weakened immune system that must be strengthened by the administration of gammaglobulin in order to fight off infections.

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

Centers for Disease Control and Prevention
1600 Clifton Road NE
Atlanta, GA 30333
Tel: (404)639-3534
Tel: (800)311-3435
Email: http://www.cdc.gov/netinfo.htm
Internet: http://www.cdc.gov/

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/

American Academy of Allergy, Asthma and Immunology
611 East Wells Street
Milwaukee, WI 53202
Tel: (414)272-6071
Fax: (414)276-3349
Tel: (800)822-2762
Email: info@aaaai.org
Internet: http://www.aaaai.org

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

Autoimmune Information Network, Inc
PO Box 4121
Brick, NJ 08723
Tel: (732)262-0450
Fax: (732)262-0450
Email: autoimmunehelp@aol.com
Internet: http://www.aininc.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

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

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: 1/9/2007
Copyright 1986, 1987, 1988, 1989, 1993, 1996, 1998, 1999, 2001, 2007 National Organization for Rare Disorders, Inc.

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