Immunodeficiency disorders
Immunosuppression; Immunodepressed - immunodeficiency; Immunosuppressed - immunodeficiency; Hypogammaglobulinemia - immunodeficiency; Agammaglobulinemia - immunodeficiencyImmunodeficiency disorders occur when the body's immune response is reduced or absent.
Immune response
The immune response is how your body recognizes and defends itself against bacteria, viruses, and substances that appear foreign and harmful....
Read Article Now Book Mark ArticleCauses
The immune system is made up of lymphoid tissue in the body, which includes:
- Bone marrow
- Lymph nodes
- Parts of the spleen and gastrointestinal tract
- Thymus
- Tonsils
Proteins and cells in the blood are also part of the immune system.
The immune system helps protect the body from harmful substances called antigens. Examples of antigens include bacteria, viruses, toxins, cancer cells, and foreign blood or tissues from another person or species.
Antigens
An antigen is any substance that causes your immune system to produce antibodies against it. This means your immune system does not recognize the su...
Read Article Now Book Mark ArticleToxins
Toxins are substances created by plants and animals that are poisonous (toxic) to humans. Toxins may also include some medicines that are helpful in...
Read Article Now Book Mark ArticleCancer
Cancer is the uncontrolled growth of abnormal cells in the body. Cancerous cells are also called malignant cells.
Read Article Now Book Mark ArticleWhen the immune system detects an antigen, it responds by activating cells known as T lymphocytes, which interact with cells known as B lymphocytes. The B cells lead to production of proteins called antibodies that destroy the harmful substances. The immune system response also involves a process called phagocytosis. During this process, certain white blood cells swallow and destroy bacteria and other foreign substances. Proteins called complement help with this process.
Antibodies
An antibody is a protein produced by the body's immune system when it detects harmful substances, called antigens. Examples of antigens include micr...
Read Article Now Book Mark ArticleImmunodeficiency disorders may affect any part of the immune system. Most often, these conditions occur when the T or B lymphocytes (or both) do not function normally or your body does not produce enough antibodies.
Inherited immunodeficiency disorders that affect B cells include:
- Hypogammaglobulinemia, which usually leads to respiratory and gastrointestinal infections
Respiratory
The words "respiratory" and "respiration" refer to the lungs and breathing.
Read Article Now Book Mark Article - Agammaglobulinemia, which results in severe infections early in life, and is often deadly
Agammaglobulinemia
Agammaglobulinemia is an inherited disorder in which a person has very low levels of protective immune system proteins called immunoglobulins. Immun...
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Inherited immunodeficiency disorders that affect T cells may cause repeated Candida (yeast) infections. Inherited combined immunodeficiency affects both T cells and B cells. It may be deadly within the first year of life if it isn't treated early.
People are said to be immunosuppressed when they have an immunodeficiency disorder due to medicines that weaken the immune system. Examples include:
- Corticosteroids
- Medicines used to treat health problems such as rheumatoid arthritis, inflammatory bowel disease, and certain skin conditions
- Chemotherapy given to treat cancer
Acquired immunodeficiency may be a complication of diseases such as HIV/AIDS and malnutrition (especially if the person does not eat enough protein). Many cancers may also cause immunodeficiency.
HIV/AIDS
Human immunodeficiency virus (HIV) is the virus that causes acquired immunodeficiency syndrome (AIDS). When a person becomes infected with HIV, the ...
Read Article Now Book Mark ArticlePeople who have had their spleen removed have an acquired immunodeficiency, and are at higher risk for infection by certain bacteria that the spleen would normally help fight. People with diabetes are also at higher risk for certain infections.
Spleen removed
Spleen removal is surgery to remove a diseased or damaged spleen. This surgery is called splenectomy. The spleen is in the upper part of the belly, ...
Read Article Now Book Mark ArticleAs you get older, the immune system becomes less effective. Immune system tissues (especially lymphoid tissue such as the thymus) shrink, and the number and activity of white blood cells drop.
The following are diseases of the immune system that can prevent it from functioning normally:
- Ataxia-telangiectasia
Ataxia-telangiectasia
Ataxia-telangiectasia is a rare childhood disease. It affects the brain and other parts of the body. Ataxia refers to uncoordinated movements, such ...
Read Article Now Book Mark Article - Complement deficiencies
- DiGeorge syndrome
- Hypogammaglobulinemia
- Job syndrome or Hyper IgE syndrome
Job syndrome
Hyperimmunoglobulin E syndrome is a rare, genetic disease. It causes problems with the skin, sinuses, lungs, bones, and teeth.
Read Article Now Book Mark Article - Leukocyte adhesion defects
- Agammaglobulinemia
- Wiskott-Aldrich syndrome
- Comon variable immunodeficiency
- Severe variable immunodeficiency
Symptoms
Your health care provider might think you have an immunodeficiency disorder if you have:
- Infections that keep coming back or do not go away
- Severe infection from bacteria or other germs that do not usually cause severe infection
Other signs include:
- Poor response to treatment for infections
- Delayed or incomplete recovery from illness
- Certain types of cancers (such as Kaposi sarcoma or non-Hodgkin lymphoma)
- Certain infections (including some forms of pneumonia or repeated yeast infections)
Symptoms depend on the disorder. For example, those with decreased levels of IgA combined with low levels of certain IgG subclasses are likely to have problems involving the lungs, sinuses, ears, throat, and digestive tract. People with disorders of their T cells also may be more likely to develop severe fungal infections.
Decreased levels of IgA
Selective deficiency of IgA is the most common immune deficiency disorder. People with this disorder have a low or absent level of a blood protein c...
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Tests used to help diagnose an immunodeficiency disorder may include:
- Complement levels in the blood, or other tests to measure substances released by the immune system
- HIV test
- Immunoglobulin levels in the blood
- Protein electrophoresis (blood or urine)
Blood
This lab test measures the types of protein in the fluid (serum) part of a blood sample. This fluid is called serum.
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The urine protein electrophoresis (UPEP) test is used to measure how much of certain proteins are in the urine.
Read Article Now Book Mark Article - T-cell lymphocyte count and B-cell lymphocyte count
T-cell lymphocyte count
A T-cell count measures the number of T cells in the blood. Your health care provider may order this test if you have signs of a weak immune system,...
Read Article Now Book Mark ArticleB-cell lymphocyte count
B-cell leukemia/lymphoma panel is a blood test that looks for certain proteins on the surface of white blood cells called B-lymphocytes. The protein...
Read Article Now Book Mark Article - White blood cell count
White blood cell count
A WBC count is a blood test to measure the number of white blood cells (WBCs) in the blood. It is a part of a complete blood count (CBC). WBCs are a...
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Treatment
The goal of treatment is to prevent infections and treat any disease and infections that do develop.
If you have a weakened immune system, you should avoid contact with persons who have infections or contagious disorders. You may have to avoid people who have been vaccinated with live virus vaccines within the past 2 weeks.
If you develop an infection, your provider will treat you aggressively. This may involve long-term use of antibiotic or antifungal drugs to prevent infections from coming back.
Interferon is used to treat viral infections and some types of cancer. It is a medicine that makes the immune system work better.
Persons with HIV/AIDS may take combinations of drugs to reduce the amount of HIV in their immune systems and improve their immunity.
People who are going to have a planned spleen removal should be vaccinated 2 weeks before the surgery against bacteria such as Streptococcus pneumonia and Haemophilus influenzae. People who haven't been previously vaccinated or have no known immunity should also receive the MMR, and chicken pox vaccines. In addition, it is also recommended that people get the DTaP vaccine series or a booster shot as needed.
MMR
All content below is taken in its entirety from the CDC MMR (Measles, Mumps, & Rubella) Vaccine Information Statement (VIS): www. cdc. gov/vaccines/h...
Read Article Now Book Mark ArticleChicken pox
All content below is taken in its entirety from the CDC Chickenpox Vaccine Information Statement (VIS): www. cdc. gov/vaccines/hcp/vis/vis-statements...
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All content below is taken in its entirety from the Centers for Disease Control and Prevention (CDC) DTaP vaccine information statement (VIS) -- www....
Read Article Now Book Mark ArticlePeople who will undergo cancer chemotherapy should get inactivated vaccines 2 weeks prior to the initiation of therapy. They should receive live vaccines 4 weeks prior to the initiation of therapy.
Bone marrow transplants may be used to treat certain immunodeficiency conditions.
Bone marrow transplants
A bone marrow transplant is a procedure to replace damaged or diseased bone marrow with healthy bone marrow stem cells. Bone marrow is the soft, fatt...
Read Article Now Book Mark ArticlePassive immunity (receiving antibodies produced by another person or animal) may sometimes be recommended to prevent illness after you have been exposed to certain bacteria or viruses.
People with low or absent levels of certain immunoglobulins may be helped with intravenous immunoglobulin (IVIG), given through a vein.
Outlook (Prognosis)
Some immunodeficiency disorders are mild and cause illness from time to time. Others are severe and may be fatal. Immunosuppression caused by medicines often goes away once the medicine is stopped.
Possible Complications
Complications of immunodeficiency disorders may include:
- Frequent or ongoing illness
- Increased risk for certain cancers or tumors
- Increased risk for infection
When to Contact a Medical Professional
Contact your provider right away if you are on chemotherapy or corticosteroids and you develop:
- A fever of 100.5°F (38°C) or higher
- A cough with shortness of breath
- Stomach pain
- Other new symptoms
Go to the emergency room or call your local emergency number (such as 911) if you have a stiff neck and headache with the fever.
Contact your provider if you have repeated yeast infections or oral thrush.
Thrush
Thrush is a yeast infection of the tongue and lining of the mouth.
Read Article Now Book Mark ArticlePrevention
There is no known way to prevent inherited immunodeficiency disorders. If you have a family history of immunodeficiency disorders, you might want to seek genetic counseling.
Practicing safer sex and avoiding the sharing of body fluids may help prevent HIV/AIDS. Ask your provider if a medicine called Truvada is right for you to prevent HIV infection.
Practicing safer sex
Safe sex means taking steps before and during sex that can prevent you from getting an infection, or from giving an infection to your partner. These...
Read Article Now Book Mark ArticleGood nutrition may prevent acquired immunodeficiency caused by malnutrition.
References
Abbas AK, Lichtman AH, Pillai S. Primary and acquired immunodeficiencies. In: Abbas AK, Lichtman AH, Pillai S, eds. Cellular and Molecular Immunology. 10th ed. Philadelphia, PA: Elsevier; 2022:chap 21.
Bonanni P, Grazzini M, Niccolai G, et al. Recommended vaccinations for asplenic and hyposplenic adult patients. Hum Vaccin Immunother. 2017;13(2):359-368. PMID: 27929751 pubmed.ncbi.nlm.nih.gov/27929751/.
Cunningham-Rundles C. Primary immunodeficiency diseases. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 231.
Antibodies - illustration
Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.
Antibodies
illustration
Antibodies - illustration
Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.
Antibodies
illustration
Review Date: 3/31/2024
Reviewed By: Deborah Pedersen, MD, MS, Allergy & Asthma Care, PC, Taunton, MA. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.