Common variable immunodeficiency
Common variable immunodeficiency: Excerpt from Professional Guide to Diseases (Eighth Edition)
Common variable immunodeficiency is characterized by progressive deterioration of B-cell (humoral) immunity, resulting in increased susceptibility to infection. Unlike X-linked hypogammaglobulinemia, this disorder (also known as acquired hypogammaglobulinemia or agammaglobulinemia with immunoglobulin [Ig]-bearing B cells) usually causes symptoms after infancy and childhood, between ages 25 and 40. It affects males and females equally and usually doesn’t interfere with normal life span or normal pregnancy and offspring.
Causes
The cause of common variable immunodeficiency is unknown. Most patients have a normal circulating B-cell count but defective synthesis or release of immunoglobulins. Many also exhibit progressive deterioration of T-cell (cell-mediated) immunity revealed by delayed hypersensitivity skin testing.
Signs and symptoms
In common variable immunodeficiency, pyogenic bacterial infections are characteristic but tend to be chronic rather than acute (as in X-linked hypogammaglobulinemia). Recurrent sinopulmonary infections, chronic bacterial conjunctivitis, and malabsorption (commonly associated with infestation by Giardia lamblia) are usually the first clues to immunodeficiency.
Common variable immunodeficiency may be associated with autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, hemolytic anemia, and pernicious anemia, and with cancers, such as leukemia and lymphoma.
Diagnosis
Characteristic diagnostic markers in this disorder are decreased serum IgM, IgA, and IgG levels detected by immunoelectrophoresis, along with a normal circulating B-cell count. Antigenic stimulation confirms an inability to produce specific antibodies; cell-mediated immunity may be intact or delayed. X-rays usually show signs of chronic lung disease or sinusitis.
Treatment
Treatment and care of patients with common variable immunodeficiency are essentially the same as for those with X-linked hypogammaglobulinemia.
Injection of immune globulin (usually weekly to monthly) helps maintain the immune response. Because these injections are painful, give them deep into a large muscle mass, such as the gluteal or thigh muscles, and massage well. If the dosage is more than 1.5 ml, divide the dose and inject it into more than one site; for frequent injections, rotate the injection sites. Because immune globulin is composed primarily of IgG, the patient may also need fresh frozen plasma infusions to provide IgA and IgM.
Antibiotics are the mainstay for combating infection. Regular X-rays and pulmonary function studies help monitor lung infection; chest physiotherapy may be ordered to forestall or help clear such infection.
Special considerations
❑ Teach the patient and his family how to recognize early signs of infection and counsel them to report such signs promptly. Advise the patient to avoid crowds and people who have active infections.
❑ Stress the importance of good nutrition and regular follow-up care.
Book Source Details
- Book Title: Professional Guide to Diseases (Eighth Edition)
- Author(s): Springhouse
- Year of Publication: 2005
- Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
» Next page: Severe combined immunodeficiency disease (Professional Guide to Diseases (Eighth Edition))
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