National Organization for Rare Disorders, Inc.

It is possible that the main title of the report Agammaglobulinemia 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.


  • hypogammaglobulinemia

Disorder Subdivisions

  • None

General Discussion

Agammaglobulinemia is a group of 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 develop or function properly leading to the deficiency in the number of mature lymphocyte cells called B cells.

The types of agammaglobulinemia are: X-linked agammaglobulinemia (XLA), the much rarer X-linked agammaglobulinemia with growth hormone deficiency (about 10 cases reported), and autosomal recessive agammaglobulinemia (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

Immune Deficiency Foundation
110 West Road
Suite 300
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)232-4636
TDD: (888)232-6348

NIH/National Institute of Allergy and Infectious Diseases
NIAID Office of Communications and Government Relations
5601 Fishers Lane, MSC 9806
Bethesda, MD 20892-9806
Tel: (301)496-5717
Fax: (301)402-3573
Tel: (866)284-4107
TDD: (800)877-8339

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

International Patient Organization for Primary Immunodeficiencies
Firside Main Road
Cornwall, PL11 3LE
United Kingdom
Tel: 441503250668
Fax: 441503250961

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

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
Fax: (732)543-7285

European Society for Immunodeficiencies
1-3 rue de Chantepoulet
Geneva, CH 1211
Tel: 410229080484
Fax: 41229069140

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 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 or email

Last Updated:  6/16/2010
Copyright  1986, 1987, 1988, 1989, 1993, 1996, 1998, 1999, 2001, 2007, 2009, 2010 National Organization for Rare Disorders, Inc.