IgA Deficiency - Janeway Type 3 Dysgammaglobulinemia
Causes: IgA deficiency seems to be linked to autosomal dominant or recessive inheritance. The presence of normal numbers of peripheral blood lymphocytes carrying IgA receptors and of normal amounts of other immunoglobulins suggests that B cells may not be secreting IgA. In an occasional patient, T-suppressor cells appear to inhibit IgA. IgA deficiency is also link to autoimmune disorders, since many patients with rheumatoid arthritis or SLE ( systemic lupus erythematosus) are also IgA deficient. Some medications, such as anticonvulsants, may cause transient IgA deficiency.
Symptoms:
May have the following signs and symptoms-
Chronic sinopulmonary
Respiratory allergy (often triggered by infection
GI tract diseases, such as celiac disease, ulcerative colitis, and regional enteritis
Autoimmune diseases, such as rheumatoid arthritis, SLE, hemolytic anemia, and chronic hepatitis
Malignant tumors, such as squamous cell carcinoma of the lungs, reticulum cell sarcoma, and thymoma
Some IgA deficient patients have no symptoms, possibly because they have extra amounts of low-molecular-weight IgM, which takes over IgA function and helps maintain immunologic defenses.
Diagnostic test:
Immunologic analyses of IgA deficient patients show serum IgA levels below 5 mg/dl. Although IgA is usually absent from secretions in IgA deficient patients, levels may be normal in rare cases.
Test may also indicate auto antibodies and antibodies against IgG (rheumatoid factor).
Treatment:
Selective IgA deficiency has no known cure. Treatment aims to control symptoms of associated diseases.
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