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Primary immunodeficiencies are a large group of hereditary or genetic disorders that impair the normal functioning of the cells of the immune system, rendering the patient unable to fight infection.
Your immune system protects your body against dangerous organisms, such as viruses, bacteria, and cancer cells. When these organisms infect an otherwise healthy body, the immune system responds by producing antibodies and special blood cells (called lymphocytes) that attempt to eradicate the infection.
When your immune system fails to respond adequately to infection, it's called an immunodeficiency, and you may be immunocompromised. People may also suffer from the opposite condition, an overactive immune system that attacks healthy cells as though they were foreign bodies, and that is called an autoimmune response. Both scenarios can create potentially life-threatening conditions.
What is an immunodeficiency disorder?
Immunodeficiency disorders prevent your body from fighting infections and diseases. This type of disorder makes it easier for you to catch viruses and bacterial infections. Immunodeficiency disorders are either congenital or acquired. A congenital, or primary, disorder is one you were born with. An acquired, or secondary, disorder is one you get later in life. Acquired disorders are more common than congenital disorders.
If you have an immunodeficiency disease, you get sick more often. Your infections may last longer and can be more serious and harder to treat. They are often genetic disorders.
Primary immunodeficiency disorders are most commonly caused by inherited gene mutations. People who have a family history of primary immunodeficiency disorders have a higher risk for developing primary disorders themselves.
Primary immunodeficiency diseases (PIDD) are a rare group of disorders that impair the immune system, causing those affected to be susceptible to chronic infections and, in some cases, having an increased risk of cancer. There are hundreds of types of PIDD.
Signs and symptoms of the conditions depend on the specific condition but can include
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