What’s hypogammaglobulinemia?

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Hypogammaglobulinemia is a disease in which the body’s immune system does not produce antibodies or produces little or no antibodies. It has a variety of causes, but the most common is a primary immune deficiency, usually the result of a genetic mutation that prevents the immune system from producing antibodies.
In a healthy immune system, cells called B lymphocytes produce antibodies in response to bacterial and viral infections. Antibodies have a variety of roles, the most important of which is to destroy invading microorganisms. This is achieved through a process called opsonization, in which antibodies coat microorganisms and make them vulnerable to destruction by other types of immune cells.

A person with hypogammaglobulinemia, therefore, is highly susceptible to bacterial infections and, to a lesser extent, viral infections. Symptoms such as chronic bacterial or viral infections such as colds, flu, tonsillitis and sinusitis are common. Chronic diarrhea, inflammatory bowel disease, weight loss, and fatigue may also occur. Children with this immune deficiency often fail to thrive and may be underweight for their age and smaller than their peers.

Many different conditions can cause antibody deficiency. One of the most common is called common variable immunodeficiency, or CVID. This immune deficiency can be caused by a number of different genetic mutations, each of which causes antibody defects that increase susceptibility to infection. Another antibody deficiency disease, called X-linked agammaglobulinemia, is a genetic disorder that is inherited on the X chromosome.

The most common treatment is regular injections of IgG immunoglobulin, also known as gamma globulin. This is an antibody preparation that is extracted from donated blood. Gamma globulin contains antibodies extracted from people who have been vaccinated or exposed to common diseases, such as mumps, chickenpox, influenza, and bacterial infections. These antibodies provide what is known as passive immunity; it is passive because it was not generated by the recipient’s immune system.

This treatment is given every three to four weeks, for the rest of the recipient’s life. It doesn’t cure hypogammaglobulinemia, but it does reduce the susceptibility to infection in people with the disorder. The recipient’s quality of life and lifespan are both greatly improved.

Treatment with gamma globulin can prevent many common infections, but not all of them can be avoided. People with antibody deficiencies generally take antibiotics even if they have a bacterial infection, no matter how mild it may be. If you have chronic infections, such as sinusitis or urinary tract infection, you may need long-term treatment with antibiotics.

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