Immunodeficiency Disorders

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. Acquired, or secondary, disorders you get later in life.
Acquired disorders are more common than congenital disorders.
Your immune system includes the following organs:
 spleen
 tonsils
 bone marrow
 lymph nodes
These organs make and release lymphocytes. These are white blood cells classified as B cells
and T cells. B and T cells fight invaders called antigens. B cells release antibodies specific to the
disease your body detects. T cells destroy foreign or abnormal cells.
Examples of antigens that your B and T cells might need to fight off include:

 bacteria
 viruses
 cancer cells
 parasites
An immunodeficiency disorder disrupts your body’s ability to defend itself against these
antigens.

What are the different types of immunodeficiency disorders?

An immune deficiency disease occurs when the immune system is not working properly. If you
are born with a deficiency or if there is a genetic cause, it is called primary immunodeficiency
disease. There are more than 100 primary immunodeficiency disorders.
Examples of primary immunodeficiency disorders include:

 X-linked agammaglobulinemia (XLA)

 common variable immunodeficiency (CVID)
 severe combined immunodeficiency (SCID), which is known as alymphocytosis or “boy in
a bubble” disease
 Secondary immunodeficiency disorders happen when an outside source like a toxic
chemical or infection attacks your body.
The following can cause a secondary immunodeficiency disorder:
 severe burns
 chemotherapy
 radiation
 diabetes
 malnutrition
Examples of secondary immunodeficiency disorders include:
 AIDS
 cancers of the immune system, like leukemia
 immune-complex diseases, like viral hepatitis
 multiple myeloma (cancer of the plasma cells, which produce antibodies)

Who is at risk for immunodeficiency disorders?

People who have a family history of primary immunodeficiency disorders have a higher-than-
normal risk for developing primary disorders.
Anything that weakens your immune system can lead to a secondary immunodeficiency
disorder. For example, exposure to bodily fluids infected with HIV, or removing the spleen can
be causes.
Spleen removal may be necessary because of conditions like cirrhosis of the liver, sickle cell
anemia, or trauma to the spleen.
Aging also weakens your immune system. As you age, some of the organs that produce white
blood cells shrink and produce fewer of them.
Proteins are important for your immunity. Not enough protein in your diet can weaken your
immune system.
Your body also produces proteins when you sleep that help your body fight infection. For this
reason, lack of sleep reduces your immune defenses. Cancers and chemotherapy drugs can also
reduce your immunity.
The following diseases and conditions are linked to primary immunodeficiency disorders:

 ataxia-telangiectasia
 Chediak-Higashi syndrome
 combined immunodeficiency disease
 complement deficiencies
 DiGeorge syndrome
 hypogammaglobulinemia
 Job syndrome
  leukocyte adhesion defects
 panhypogammaglobulinemia
 Bruton’s disease
 congenital agammaglobulinemia
 selective deficiency of IgA
 Wiskott-Aldrich syndrome

Signs of an immunodeficiency disorder

Each disorder has unique symptoms that can be frequent or chronic. Some of these symptoms
can include:
 pinkeye
 sinus infections
 colds
 diarrhea
 pneumonia
 yeast infections
If these problems don’t respond to treatment or you don’t completely get better over time,
your doctor might test you for an immunodeficiency disorder.

How are immune disorders diagnosed?

If your doctor thinks you might have an immunodeficiency disorder, they will want to do the
following:

 ask you about your medical history

 perform a physical exam
 determine your white blood cell count
 determine your T cell count
 determine your immunoglobulin levels
Vaccines can test your immune system response in what is called an antibody test. Your doctor
will give you a vaccine. Then they will test your blood for its response to the vaccine a few days
or weeks later.
If you don’t have an immunodeficiency disorder, your immune system will produce antibodies
to fight the organisms in the vaccine. You might have a disorder if your blood test doesn’t show
antibodies.

How are immunodeficiency disorders treated?

The treatment for each immunodeficiency disorder will depend on the specific conditions. For
example, AIDS causes several different infections. Your doctor will prescribe medications for
each infection. And you may be given an antiretroviral to treat and HIV infection if appropriate.
Treatment for immunodeficiency disorders commonly includes antibiotics and immunoglobulin
therapy. Other antiviral drugs, amantadine and acyclovir, or a drug called interferon are used
for treatment of the viral infections caused by immunodeficiency disorders.
If your bone marrow isn’t producing enough lymphocytes, your doctor might order a bone
marrow (stem cell) transplant.