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Children's immunologist

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General Variable Immunodeficiency: Causes, Symptoms, Diagnosis, Treatment

Alexey Portnov, medical expert
Last reviewed: 23.04.2024

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The total variable immunodeficiency (acquired hypogammaglobulinemia or hypogammaglobulinemia late-onset) is characterized by a low level of lg with phenotypically normal B lymphocytes that are capable of proliferation but do not complete their development by the formation of Ig-producing cells.

The total variable immunodeficiency (OID) includes several different molecular defects, but in most patients, molecular defects are not known. According to the types of developing infectious processes, OID is clinically similar to agammaglobulinemia linked to the X-chromosome, but begins later, sometimes in adulthood. In some patients, the T-cell immune response may be compromised. There are autoimmune diseases (for example, SLE, Addison's disease, thyroiditis, rheumatoid arthritis, alopecia areata, autoimmune hemolytic or pernicious anemia), as well as malabsorption, nodular lymphoid hyperplasia of the gastrointestinal tract, lymphoid interstitial pneumonia, splenomegaly, bronchiectasis. In 10% of patients, carcinoma of the stomach and lymphoma are detected.

The diagnosis is based on the presence in the family history of autoimmune diseases and is confirmed by the measurement of serum Ig and titer antibodies to protein and polysaccharide vaccine antigens. If the level in both cases is low, then B-lymphocytes are counted by flow cytometry for differential diagnosis of the total variable immunodeficiency and agammaglobulimemia linked to the X-chromosome, multiple myeloma or chronic lymphocytic leukemia. Whey protein electrophoresis will help to detect monoclonal gammopathies (eg, myeloma), which are accompanied by a decreased level of lg or other lg-isotypes. Treatment consists of intravenous immunoglobulin 400 mg / kg / month and antibiotic therapy for the treatment of infections.

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