Primary Agammaglobulinemias / Disease

Diseases & Conditions

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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
Firside
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