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Agamaglobulinesia! Say what?????????

Agammaglobulinemia

Agammaglobulinemia is an inherited disorder in which a person has very 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
This is a rare disorder that mainly affects males. It is caused by a gene defect that blocks the growth of normal, mature immune cells called B lymphocytes.
As a result, the body makes very little (if any) immunoglobulins. Immunoglobulins play a major role in the immune response, which protects against illness and infection.
People with this disorder develop infections again and again. Common infections include ones that are due to bacteria such as Haemophilus 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 (airway infection)
Chronic diarrhea
Conjunctivitis (eye infection)
Otitis media (middle ear infection)
Pneumonia (lung infection)
Sinusitis (sinus infection)
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)
Asthma without a known cause
Exams and Tests
The disorder is confirmed by blood tests that measure levels of 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. Antibiotics are often needed to treat bacterial infections.
Immunoglobulins are given through a vein or by injection to boost the immune system.
A bone marrow transplant may be considered.
Outlook (Prognosis)
Treatment with immunoglobulins has greatly improved the health of those who have this disorder.
Without treatment, most severe infections are deadly.
Possible Complications
Health problems that may result include:
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.
Alternative Names
Bruton’s agammaglobulinemia; X-linked agammaglobulinemia; Immunosuppression – agammaglobulinemia; Immunodepressed – agammaglobulinemia; Immunosuppressed – agammaglobulinemia
Images
Antibodies
References
Buckley RH. Primary defects of antibody production. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 124.
Cunningham-Rundles C. Primary immunodeficiency diseases. In: Goldman L, Schafer AI, eds. Goldman’s Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 250.
Pai SY, Notarangelo LD. Congenital disorders of lymphocyte function. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 49.
Review Date 3/20/2016
Updated by: Stuart I. Henochowicz, MD, FACP, Associate Clinical Professor of Medicine, Division of Allergy, Immunology, and Rheumatology, Georgetown University Medical School, Washington, DC. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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