St. Luke's Hospital
Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
Find a Physician Payment Options Locations & Directions
Follow us on: facebook twitter Mobile Email Page Email Page Print Page Print Page Increase Font Size Decrease Font Size Font Size
America's 50 Best Hospitals
Meet the Doctor
Spirit of Women
Community Health Needs Assessment
Home > Health Information

Multimedia Encyclopedia

    Print-Friendly
    Bookmarks

    Agammaglobulinemia

    Bruton's agammaglobulinemia; X-linked agammaglobulinemia

    Agammaglobulinemia is disorder passed down through families in which a person hasvery low levels of protective immune system proteins called immunoglobulins. Immunoglobulins are a type of antibody. Low levels of these antibodies make you more likely to get infections.

    Causes

    Agammaglobulinemia is a rare disorder that mainly affects males. It is caused by a gene defectthat blocks the growth of normal, mature immune cells called B lymphocytes.

    As a result, the body makes very little (if any) immunoglobulins in the bloodstream. Immunoglobulins play a major role in the immune response, which protects against illness and infection.

    Persons with agammaglobulinemia repeatedly develop infections, especially bacterial infections such as Hemophilus influenzae, pneumococci (Streptococcus pneumoniae), and staphylococci. Common sites of infection include:

    • Gastrointestinal tract
    • Joints
    • Lungs
    • Skin
    • Upper respiratory tract

    Agammaglobulinemia is inherited, which means other people in your family may have the condition.

    Symptoms

    Symptoms include frequent episodes of:

    • Bronchitis
    • Chronic diarrhea
    • Conjunctivitis (eye infection)
    • Otitis media (middle ear infection)
    • Pneumonia
    • Sinusitis
    • Skin infections
    • Upper respiratory tract infections

    Infections typically appear in the first 4 years of life.

    Other symptoms include:

    • Bronchiectasis (a disease in which the small air sacs in the lungs become damaged and enlarged)
    • Unexplained asthma

    Exams and Tests

    The disorder is confirmed by laboratory measurement of blood immunoglobulins.

    Tests include:

    • Flow cytometry to measure circulating B lymphocytes
    • Immunoelectrophoresis - serum
    • Quantitative immunoglobulins - IgG, IgA, IgM (usually measured by nephelometry)

    Treatment

    Treatment involves taking steps to reduce the number and severity of infections. You will receive immunoglobulins through a vein (IVIG), which boosts your immune system.

    Antibiotics are often needed to treat bacterial infections.

    Outlook (Prognosis)

    Treatment with IVIG has greatly improved the health of those who have agammaglobulinemia.

    Without treatment, most severe infections are deadly.

    Possible Complications

    • Arthritis
    • Chronic sinus or pulmonary disease
    • Eczema
    • Intestinal malabsorption syndromes

    When to Contact a Medical Professional

    Call for an appointment with your health care provider if:

    • You or your child has experienced frequent infections
    • You have a family history of agammaglobulinemia or another immunodeficiency disorder and you are planning to have children (ask the provider about genetic counseling)

    Prevention

    Genetic counseling should be offered to prospective parents with a family history of agammaglobulinemia or other immunodeficiency disorders.

    References

    Ballow M. Primary immunodeficiency diseases. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier;2011:chap 258.

    Morimoto Y. Immunodeficiency overview. Prim Care. 2008;35(1):159-173.

    BACK TO TOP

    • Antibodies

      illustration

      • Antibodies

        illustration

      Tests for Agammaglobulinemia

        Review Date: 5/26/2012

        Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Stuart I. Henochowicz, MD, FACP, Associate Clinical Professor of Medicine, Division of Allergy, Immunology, and Rheumatology, Georgetown University Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.

        The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
        adam.com

        A.D.A.M. content is best viewed in IE9 or above, Fire Fox and chrome browser.


        Back  |  Top
        About Us
        Contact Us
        History
        Mission
        Locations & Directions
        Quality Reports
        Annual Reports
        Honors & Awards
        Community Health Needs
        Assessment

        Newsroom
        Services
        Brain & Spine
        Cancer
        Heart
        Maternity
        Orthopedics
        Pulmonary
        Sleep Medicine
        Urgent Care
        Women's Services
        All Services
        Patients & Visitors
        Locations & Directions
        Find a Physician
        Tour St. Luke's
        Patient & Visitor Information
        Contact Us
        Payment Options
        Financial Assistance
        Send a Card
        Mammogram Appointments
        Health Tools
        My Personal Health
        mystlukes
        Spirit of Women
        Health Information & Tools
        Clinical Trials
        Health Risk Assessments
        Employer Programs -
        Passport to Wellness

        Classes & Events
        Classes & Events
        Spirit of Women
        Donate & Volunteer
        Giving Opportunities
        Volunteer
        Physicians & Employees
        For Physicians
        Remote Access
        Medical Residency Information
        Pharmacy Residency Information
        Physician CPOE Training
        Careers
        Careers
        St. Luke's Hospital - 232 South Woods Mill Road - Chesterfield, MO 63017 Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
        Copyright © St. Luke's Hospital Website Terms and Conditions  |  Privacy Policy  |  Patient Notice of Privacy Policies PDF Sitemap St. Luke's Mobile