Hypogammaglobulinemia is an immune deficiency disorder and is characterized by a low blood level of antibodies (immunoglobulins), specifically immunoglobulin type G (IgG), resulting in an increased susceptibility to recurring infections. Hypogammaglobulinemia can be of primary or secondary origin. Primary immune deficiencies result from genetic disorders and/or chromosomal anomalies during the development of the immune system. Secondary causes are caused by an external or acquired factor such as a corticosteroid or immunosuppressant drug, nutritional disorders, infections, chemotherapy, malignancy, nephrotic syndrome, other metabolic diseases, and hazardous environmental conditions. It is important that physicians distinguish between primary and secondary causes of hypogammaglobulinemia in order to provide appropriate treatment. People diagnosed with non-specific types of immune deficiencies may not meet the strict diagnostic criteria for other deficiencies (e.g., common variable immunodeficiency [CVID]) but still have low levels of IgG and lack of functional antibodies.
Symptoms of Hypogammaglobulinemia
The usual presenting feature of hypogammaglobulinemia is recurrent infections.
Various laboratory and imaging studies may be helpful in diagnosing hypogammaglobulinemia as well as determining a secondary cause. Having a low IgG level may not always be related to immune deficiency but may be caused by another condition such as a kidney or gastrointestinal condition, cancer or severe burns, or may be related to medication such as long term use of corticosteroids.
If the cause of hypogammaglobulinemia is secondary, treating the underlying cause may correct the problem. When other conditions or medications causing hypogammaglobulinemia are ruled out, patients can receive immune globulin replacement therapy. Immune globulin is extracted from a large pool of human plasma and contains all the important antibodies present in the normal population. Immune globulin therapy can be administered into the blood stream intravenously (IVIG) or under the skin subcutaneously (SCIG).
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