Diseases & Conditions


Primary Agammaglobulinemias

Synonyms of Primary Agammaglobulinemias
  • Antibody Deficiency
  • Gammaglobulin Deficiency
  • Immunoglobulin Deficiency

Disorder Subdivisions

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

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.

Organizations related to Primary Agammaglobulinemias
  • American Academy of Allergy, Asthma and Immunology
    611 East Wells Street
    Milwaukee WI 53202
    Phone #: 414-272-6071
    800 #: 800-822-2762
    e-mail: [email protected]
    Home page: http://www.aaaai.org
  • Autoimmune Information Network, Inc
    PO Box 4121
    Brick NJ 08723
    Phone #: 732-664-9259
    800 #: N/A
    e-mail: [email protected]
    Home page: http://www.aininc.org
  • Centers for Disease Control and Prevention
    1600 Clifton Road NE
    Atlanta GA 30333
    Phone #: 404-639-3534
    800 #: 800-311-3435
    e-mail: http://www.cdc.gov/netinfo.htm
    Home page: http://www.cdc.gov/
  • 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: [email protected]
    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: [email protected]
    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: [email protected]
    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: [email protected]
    Home page: http://www.info4pi.org
  • MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
    150 Custer Court
    Green Bay WI 54301-1243
    Phone #: 920-336-5333
    800 #: 877-336-5333
    e-mail: [email protected]
    Home page: http://www.netnet.net/mums/
  • Madisons Foundation
    PO Box 241956
    Los Angeles CA 90024
    Phone #: 310-264-0826
    800 #: N/A
    e-mail: [email protected]
    Home page: http://www.madisonsfoundation.org
  • March of Dimes Birth Defects Foundation
    1275 Mamaroneck Avenue
    White Plains NY 10605
    Phone #: 914-428-7100
    800 #: 888-663-4637
    e-mail: [email protected]
    Home page: http://www.marchofdimes.com
  • 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/

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