Immunodeficiency Disorders

Written by Elea Carey | Published on August 29, 2012
Medically Reviewed by George Krucik, MD

What Is an Immunodeficiency Disorder?

Immunodeficiency disorders prevent your body from being able to fight infections and diseases the way it should. An immunodeficiency disorder makes you considerably more susceptible to catching viruses and bacterial infections.

Immune disorders are oftentimes categorized as either congenital or acquired. When you’re born with a disorder, it’s sometimes called a congenital or primary disorder. Acquired disorders are sometimes called secondary disorders. Secondary disorders are more common than primary (UpToDate, 2012).

How Does the Immune System Work?

To understand immune disorders, you need to understand the basics of how your immune system works. The immune system is made up of several organs including your spleen, tonsils, bone marrow, and lymph nodes. These organs make and release lymphocytes, which are white blood cells classified as B cells and T cells. B and T cells fight invaders called antigens. Examples of antigens are bacteria, viruses, cancer cells, or bodily fluids from a sick person. B cells release antibodies specific to the disease your body detects. T cells kill off the cells in your body that are being attacked by disease.

What Are the Different Types of Immunodeficiency Disorders?

Primary immunodeficiency disorders are immune disorders you are born with. Primary disorders include:

  • X-linked agammaglobulinemia (XLA)
  • severe combined immunodeficiency (SCID disorders)
  • common variable immunodeficiency
  • Alymphocytosis (“boy in a bubble” disease)

Secondary disorders happen when your body is attacked by an outside source, such as a toxic chemical or an infection. Severe burns and radiation also can cause secondary disorders. Secondary disorders include:

  • AIDS
  • cancers of the immune system, such as leukemia
  • immune complex diseases, such as viral hepatitis
  • multiple myeloma

Who Is at Risk For Immunodeficiency Disorders?

People who have a family history of primary disorders are at a higher-than-normal risk for them.

Anything that weakens your immune system can lead to a secondary immunodeficiency disorder. For example, the exposure to bodily fluids such as blood and sperm from someone infected with HIV can cause AIDS.

Removing the spleen can weaken your immune system. Spleen removal may be required in response to one of any number of diseases or injuries, including 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 to your immunity. An insufficient amount of protein in the diet can reduce the strength of your immune system. Additionally, when you sleep, your body produces proteins that help your immune system fight infection. For this reason, lack of sleep reduces your immune defenses.

Cancers and the chemotherapy drugs used to treat them can reduce your immunity.

The following diseases and conditions are also linked to 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

Recognizing the Signs of an Immunodeficiency Disorder

Each disorder has unique symptoms. The first indicator that something is wrong with your immune system is becoming repeatedly and chronically sick, even with minor illnesses, including pinkeye, sinus infections, or diarrhea. If these problems don’t respond to treatment or don’t completely get better over time, your doctor might test you for a disorder. Recurrent pneumonia and yeast infections could also suggest you have a disorder.

How Are Immune Disorders Diagnosed?

If your doctor thinks you might have an immunodeficiency disorder, he or she will want to do a thorough medical exam including:

  • medical history
  • physical exam
  • T cell count
  • white blood cell count

Vaccines can be used to test your immune system response in what is called an antibody test. Your doctor will give you a vaccine and make an appointment in a few days or weeks to test your blood for its response to the vaccine. 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?

Each disorder will be treated according to the specific conditions it causes. For example, AIDS causes several different infections, including Kaposi’s sarcoma, which is treated with doxorubicin lipid complex, and cryptococcosis, which is treated with fluconazole.

Treatment for immunodeficiency disorders commonly includes antibiotics and antibody replacement. A drug called interferon is often used to treat the viral infections caused by a disorder.

If your bone marrow is not producing enough lymphocytes, your doctor might order a bone marrow transplant.

What Is the Outlook for Someone With an Immunodeficiency Disorder?

Most doctors agree that people with immunodeficiency disorders can lead full and productive lives. Early identification and treatment of the disorder and the problems it causes are very important.

How Can Immunodeficiency Disorders Be Prevented?

Primary disorders can be controlled and treated, but not prevented. Secondary disorders can be prevented in a number of ways. For example, it is possible to avoid AIDS by not having unprotected sex with someone who carries the virus.

Sleep is very important to a healthy immune system. According to the Mayo Clinic, adults need about eight hours of sleep per night (Mayo Clinic, 2012).

If your immune system is not working properly, it is important that you stay away from people who are sick.

If you have a contagious immune disorder like AIDS, you can keep others healthy by practicing safe sex and not sharing bodily fluids with people who don’t have the condition.

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