Primary Agammaglobulinemias

National Organization for Rare Disorders, Inc.

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


  • 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.


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

Immune Deficiency Foundation
40 West Chesapeake Avenue
Suite 308
Towson, MD 21204
Tel: (410)321-6647
Fax: (410)321-9165
Tel: (800)296-4433

Centers for Disease Control and Prevention
1600 Clifton Road NE
Atlanta, GA 30333
Tel: (404)639-3534
Tel: (800)311-3435

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

American Academy of Allergy, Asthma and Immunology
555 East Wells Street, Suite 1100
Milwaukee, WI 53202-3823
Tel: (414)272-6071
Fax: (414)276-3349
Tel: (800)822-2762

International Patient Organization for Primary Immunodeficiencies
Main Road
Cornwall, PL11 3LE
United Kingdom
Tel: 44 1503 250 668
Fax: 44 1503 250 668

Jeffrey Modell Foundation
747 Third Avenue
34th Floor
New York, NY 10017
Tel: (212)819-0200
Fax: (212)764-4180
Tel: (866)469-6474

MUMS National Parent-to-Parent Network
150 Custer Court
Green Bay, WI 54301-1243
Tel: (920)336-5333
Fax: (920)339-0995
Tel: (877)336-5333

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

Madisons Foundation
PO Box 241956
Los Angeles, CA 90024
Tel: (310)264-0826
Fax: (310)264-4766

Autoimmune Information Network, Inc
PO Box 4121
Brick, NJ 08723
Tel: (732)664-9259

European Society for Immunodeficiencies (ESID)
c/o Dr. Bodo Grimbacher (ESID Board Member)
Division of Rheumatology and Clinical Immunology
Department of Medicine, University Hospital Freiburg
Hugstetter Strasse 55
Freiburg, D-79106
Tel: +31 73-6992965
Fax: +31 73-6992948

For a Complete Report

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

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