As its name implies, immunodeficiency disorders are disorders that interfere with or affect immune system function. When this happens, the body becomes unable to fight off foreign invaders, such as bacteria, viruses, illnesses and other substances. As a result, people who are immunocompromised are more susceptible to infections and illnesses, including but not limited to lung infections, ear infections, gastrointestinal issues, sinus problems and more. For people with more severe immunodeficiencies, such disorders can have serious and possibly life-threatening complications.
There are more than 400 genetic immunodeficiency disorders a person can have. These disorders do not take into account the hundreds more disorders that are caused by environmental or external factors. Each of these disorders affect the body in different ways, making it difficult for researchers and healthcare providers to explain how, exactly, immunodeficiency affects the immune system. That said, scientists have attempted to come up with a generalized explanation.
The immune system is your body’s defence against foreign invaders that may or may not cause damage to it and/or hinder its functions. The immune system is responsible for identifying invaders before they have a chance to replicate and/or destroy them before they have an opportunity to cause considerable damage. Said system is made of up two components: innate and adaptive immunities.
Innate immunity is your body’s first line of defence against viruses, bacteria and fungi. Innate immunity prevents foreign invaders from entering the body entirely by blocking them off at the skin and mucous membranes. It does this with the help of certain types of white blood cells, which surround, ingest and kill bacteria or viruses before they have a chance to enter the blood stream.
The cells that make up the innate immunity barrier also activate a more precise and long-lived immune response known as adaptive immunity. Adaptive immunity is made up of T cells, which either trigger the production of pathogen antibodies or attack infectious agents directly.
For the immune system to function properly, it requires the help of several bodily components, right down to the bone marrow. In fact, the bone marrow produces and houses B cells until they mature. B cells are almost wholly responsible for the effective functioning of the immune system. The thymus also plays an important role, housing T cells until they, too, mature.
Once matured, both B cells and T cells move to the lymph nodes and spleen, where they wait for other cells to signal the need to fight an infection. They also have the freedom to move to other areas of the body if the need arises. Some other areas of the body to which immune cells might travel are as follows:
If the B or T cells travel to the tonsils, appendix or certain parts of the small intestine, they may end up in special tissue designed to house them until they are needed.
Finally, the immune system consists of the complement system, which is responsible for activating more than 30 different kinds of proteins in the body. When they encounter pathogens, these proteins help the immune system as a whole launch an attack.
When a person lives with an immunodeficiency disorder, he or she may experience problems with one or more components of his or her immune system. For some people, these issues may never manifest in noticeable symptoms. For others, it may take years for issues to become apparent. Lastly, for others, an immunodeficiency can cause so many and/or such severe complications that immediate and ongoing treatment are necessary for their health and well-being. Regardless of how severe a deficiency is, however, the bottom line is that, with an immunodeficiency disorder, the immune system fails to function properly.
Once upon a time, scientists considered immunodeficiency disorders to be rare. Today, however, thanks to advanced diagnostic tools and medical understanding, it is becoming evident that such disorders are more widespread than they initially believed. According to the data, as many as one in every 1,200 to 2,000 people worldwide could have a primary immunodeficiency disorder. This data does not account for secondary immunodeficiency disorders, which are far more common than PIDs.
Autoimmune Lymphoproliferative Syndrome (ALPS)
Caspase Eight Deficiency State (CEDS)
CARD9 Deficiency And Other Syndromes Of Susceptibility To Candidiasis
Chronic Granulomatous Disease (CGD)
Common Variable Immunodeficiency (CVID)
Congenital Neutropenia Syndromes
Hyper-Immunoglobulin E Syndromes
Hyper-Immunoglobulin M Syndromes
Interferon Gamma, Interleukin 12 And Interleukin 23 Deficiencies
Leukocyte Adhesion Deficiency (LAD)
PLCG2-Associated Antibody Deficiency And Immune Dysregulation (PLAID)
Severe Combined Immunodeficiency (SCID)
STAT3 Dominant Negative Disease (STAT3DN)
STAT3 Gain-Of-Function Disease (STAT3 GOF)
Warts, Hypogammaglobulinemia, Infections, And Myelokathexis (WHIM) Syndrome
Wiskott-Aldrich Syndrome (WAS)
X-Linked Agammaglobulinemia (XLA)
X-Linked Lymphoproliferative Disease (XLP)
How an immunodeficiency disorder affects the immune system depends largely on the immune system in question. However, disorders impede or restrict immune system function, making it difficult for the immune system to do its primary job, which is to fight off foreign invaders and keep the body healthy. Some such disorders are easily remedied while others require ongoing care and management. That said, the key to a successful outcome lies with obtaining an accurate diagnosis from a qualified healthcare professional.