Primary Immune Deficiency Overview

There are two types of immune deficiency diseases. Primary immune deficiency is when the body's immune defense system is missing a part or does not work correctly. This condition is caused by a genetic problem in the body's defense system. Secondary immune deficiency disease is when the body's defense system has a problem caused by outside factors such as chemotherapy or viruses.

The immune system is very complicated. B-cells (B-lymphocytes) develop from cells inside the bone, lymph nodes, spleen, some parts of the intestine and a small amount in the blood. Foreign materials (antigens), such as germs, cause the B-cells to work. They grow and change into plasma cells that produce antibodies. Antibodies and antigens fit together like a lock and key and attach to each other, which activates the immune system to destroy the foreign material. Immune globulins or gamma globulins are another name for antibody proteins.

There are almost 100 different primary immune deficiency diseases. Some occur as often as 1 in 500 to 1 in a million. If a primary immune deficiency is left without treatment it causes very serious illness and life-threatening infections.

Today, doctors know the signs of primary immune disease so that people who may not have grown up to be adults in the past are now living close to normal lives with treatment. Many patients with primary immune deficiency will need treatment with intravenous gamma globulin infusions, antibiotic medications, or bone marrow transplantation. Gamma globulin, one of the treatments, is extracted from healthy people's donation of plasma and is purified through a manufacturing process.

Living with Immune Deficiency

If you are on immuneglobulin therapy:

Learning about your treatment is important so that you can take part in your every day life. To learn more about Immune Deficiency, please visit: www.primaryimmune.org, www.niaid.nih.gov/final/immds/immdef.htm or www.immunedisease.com.

Healthy Pointers:

Questions? Call us at 1.888.282.5166 or email us.


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