Immune System (page 2)

The immune system, which is made up of special cells, proteins, tissues, and organs, defends people against germs and microorganisms every day. In most cases, the immune system does a great job of keeping people healthy and preventing infections. But sometimes problems with the immune system can lead to illness and infection.

About the Immune System

The immune system is the body's defense against infectious organisms and other invaders. Through a series of steps called the immune response, the immune system attacks organisms and substances that invade body systems and cause disease.

The immune system is made up of a network of cells, tissues, and organs that work together to protect the body. The cells involved are white blood cells, or leukocytes, which come in two basic types that combine to seek out and destroy disease-causing organisms or substances.

Leukocytes are produced or stored in many locations in the body, including the thymus, spleen, and bone marrow. For this reason, they're called the lymphoid organs. There are also clumps of lymphoid tissue throughout the body, primarily as lymph nodes, that house the leukocytes.

The leukocytes circulate through the body between the organs and nodes via lymphatic vessels and blood vessels. In this way, the immune system works in a coordinated manner to monitor the body for germs or substances that might cause problems.

The two basic types of leukocytes are:

1. phagocytes, cells that chew up invading organisms
2. lymphocytes, cells that allow the body to remember and recognize previous invaders and help the body destroy them

A number of different cells are considered phagocytes. The most common type is the neutrophil, which primarily fights bacteria. If doctors are worried about a bacterial infection, they might order a blood test to see if a patient has an increased number of neutrophils triggered by the infection. Other types of phagocytes have their own jobs to make sure that the body responds appropriately to a specific type of invader.

The two kinds of lymphocytes are B lymphocytes and T lymphocytes. Lymphocytes start out in the bone marrow and either stay there and mature into B cells, or they leave for the thymus gland, where they mature into T cells. B lymphocytes and T lymphocytes have separate functions: B lymphocytes are like the body's military intelligence system, seeking out their targets and sending defenses to lock onto them. T cells are like the soldiers, destroying the invaders that the intelligence system has identified.

Here's how it works:

When antigens (foreign substances that invade the body) are detected, several types of cells work together to recognize them and respond. These cells trigger the B lymphocytes to produce antibodies, specialized proteins that lock onto specific antigens.

Once produced, these antibodies continue to exist in a person's body, so that if the same antigen is presented to the immune system again, the antibodies are already there to do their job. So if someone gets sick with a certain disease, like chickenpox, that person typically doesn't get sick from it again.

This is also how immunizations prevent certain diseases. An immunization introduces the body to an antigen in a way that doesn't make someone sick, but does allow the body to produce antibodies that will then protect the person from future attack by the germ or substance that produces that particular disease.

Although antibodies can recognize an antigen and lock onto it, they are not capable of destroying it without help. That's the job of the T cells, which are part of the system that destroys antigens that have been tagged by antibodies or cells that have been infected or somehow changed. (Some T cells are actually called "killer cells.") T cells also are involved in helping signal other cells (like phagocytes) to do their jobs.

Antibodies also can neutralize toxins (poisonous or damaging substances) produced by different organisms. Lastly, antibodies can activate a group of proteins called complement that are also part of the immune system. Complement assists in killing bacteria, viruses, or infected cells.

All of these specialized cells and parts of the immune system offer the body protection against disease. This protection is called immunity.

Immunodeficiency Disorders

Immunodeficiencies occur when a part of the immune system is not present or is not working properly. Sometimes a person is born with an immunodeficiency (known as primary immunodeficiencies), although symptoms of the disorder might not appear until later in life. Immunodeficiencies also can be acquired through infection or produced by drugs (these are sometimes called secondary immunodeficiencies).

Immunodeficiencies can affect B lymphocytes, T lymphocytes, or phagocytes. Examples of primary immunodeficiencies that can affect kids and teens are:

• IgA deficiency is the most common immunodeficiency disorder. IgA is an immunoglobulin that is found primarily in the saliva and other body fluids that help guard the entrances to the body. IgA deficiency is a disorder in which the body doesn't produce enough of the antibody IgA. People with IgA deficiency tend to have allergies or get more colds and other respiratory infections, but the condition is usually not severe.
• Severe combined immunodeficiency (SCID) is also known as the "bubble boy disease" after a Texas boy with SCID who lived in a germ-free plastic bubble. SCID is a serious immune system disorder that occurs because of a lack of both B and T lymphocytes, which makes it almost impossible to fight infections.
• DiGeorge syndrome (thymic dysplasia), a birth defect in which kids are born without a thymus gland, is an example of a primary T-lymphocyte disease. The thymus gland is where T lymphocytes normally mature.
• Chediak-Higashi syndrome and chronic granulomatous disease both involve the inability of the neutrophils to function normally as phagocytes.

Acquired (or secondary) immunodeficiencies usually develop after someone has a disease, although they can also be the result of malnutrition, burns, or other medical problems. Certain medicines also can cause problems with the functioning of the immune system.

Acquired (secondary) immunodeficiencies include:

• HIV (human immunodeficiency virus) infection/AIDS (acquired immunodeficiency syndrome) is a disease that slowly and steadily destroys the immune system. It is caused by HIV, a virus that wipes out certain types of lymphocytes called T-helper cells. Without T-helper cells, the immune system is unable to defend the body against normally harmless organisms, which can cause life-threatening infections in people who have AIDS. Newborns can get HIV infection from their mothers while in the uterus, during the birth process, or during breastfeeding. People can get HIV infection by having unprotected sexual intercourse with an infected person or from sharing contaminated needles for drugs, steroids, or tattoos.
• Immunodeficiencies caused by medications. Some medicines suppress the immune system. One of the drawbacks of chemotherapy treatment for cancer, for example, is that it not only attacks cancer cells, but other fast-growing, healthy cells, including those found in the bone marrow and other parts of the immune system. In addition, people with autoimmune disorders or who have had organ transplants may need to take immunosuppressant medications, which also can reduce the immune system's ability to fight infections and can cause secondary immunodeficiency.

Autoimmune Disorders

In autoimmune disorders, the immune system mistakenly attacks the body's healthy organs and tissues as though they were foreign invaders. Autoimmune diseases include:

• Lupus, a chronic disease marked by muscle and joint pain and inflammation (the abnormal immune response also may involve attacks on the kidneys and other organs)
• Juvenile rheumatoid arthritis, a disease in which the body's immune system acts as though certain body parts (such as the joints of the knee, hand, and foot) are foreign tissue and attacks them
• Scleroderma, a chronic autoimmune disease that can lead to inflammation and damage of the skin, joints, and internal organs
• Ankylosing spondylitis, a disease that involves inflammation of the spine and joints, causing stiffness and pain
• Juvenile dermatomyositis, a disorder marked by inflammation and damage of the skin and muscles

Allergic Disorders

Allergic disorders occur when the immune system overreacts to exposure to antigens in the environment. The substances that provoke such attacks are called allergens. The immune response can cause symptoms such as swelling, watery eyes, and sneezing, and even a life-threatening reaction called anaphylaxis. Medications called antihistamines can relieve most symptoms.

Allergic disorders include:

• Asthma, a respiratory disorder that can cause breathing problems, frequently involves an allergic response by the lungs. If the lungs are oversensitive to certain allergens (like pollen, molds, animal dander, or dust mites), it can trigger breathing tubes in the lungs to become narrowed and swollen, leading to reduced airflow and making it hard for a person to breathe.
• Eczema is an itchy rash also known as atopic dermatitis. Although atopic dermatitis is not necessarily caused by an allergic reaction, it more often occurs in kids and teens who have allergies, hay fever, or asthma or who have a family history of these conditions.
• Allergies of several types can occur in kids and teens. Environmental allergies (to dust mites, for example), seasonal allergies (such as hay fever), drug allergies (reactions to specific medications or drugs), food allergies (such as to nuts), and allergies to toxins (bee stings, for example) are the common conditions people usually refer to as allergies.

Cancers of the Immune System

Cancer occurs when cells grow out of control. This also can happen with the cells of the immune system. Leukemia, which involves abnormal overgrowth of leukocytes, is the most common childhood cancer. Lymphoma involves the lymphoid tissues and is one of the more common childhood cancers. With current medications most cases of both types of cancer in kids and teens are curable.

Although immune system disorders usually can't be prevented, you can help your child's immune system stay stronger and fight illnesses by staying informed about your child's condition and working closely with your doctor.

Reviewed by: Yamini Durani, MD
Date reviewed: October 2012