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By the end of this section, you will be able to:
  • Discuss inherited and acquired immunodeficiencies
  • Explain the four types of hypersensitivity and how they differ
  • Give an example of how autoimmune disease breaks tolerance

This section is about how the immune system goes wrong. When it goes haywire, and becomes too weak or too strong, it leads to a state of disease. The factors that maintain immunological homeostasis are complex and incompletely understood.

Immunodeficiencies

As you have seen, the immune system is quite complex. It has many pathways using many cell types and signals. Because it is so complex, there are many ways for it to go wrong. Inherited immunodeficiencies arise from gene mutations that affect specific components of the immune response. There are also acquired immunodeficiencies with potentially devastating effects on the immune system, such as HIV.

Inherited immunodeficiencies

A list of all inherited immunodeficiencies is well beyond the scope of this book. The list is almost as long as the list of cells, proteins, and signaling molecules of the immune system itself. Some deficiencies, such as those for complement, cause only a higher susceptibility to some Gram-negative bacteria. Others are more severe in their consequences. Certainly, the most serious of the inherited immunodeficiencies is severe combined immunodeficiency disease (SCID)    . This disease is complex because it is caused by many different genetic defects. What groups them together is the fact that both the B cell and T cell arms of the adaptive immune response are affected.

Children with this disease usually die of opportunistic infections within their first year of life unless they receive a bone marrow transplant. Such a procedure had not yet been perfected for David Vetter, the “boy in the bubble,” who was treated for SCID by having to live almost his entire life in a sterile plastic cocoon for the 12 years before his death from infection in 1984. One of the features that make bone marrow transplants work as well as they do is the proliferative capability of hematopoietic stem cells of the bone marrow. Only a small amount of bone marrow from a healthy donor is given intravenously to the recipient. It finds its own way to the bone where it populates it, eventually reconstituting the patient’s immune system, which is usually destroyed beforehand by treatment with radiation or chemotherapeutic drugs.

New treatments for SCID using gene therapy, inserting nondefective genes into cells taken from the patient and giving them back, have the advantage of not needing the tissue match required for standard transplants. Although not a standard treatment, this approach holds promise, especially for those in whom standard bone marrow transplantation has failed.

Human immunodeficiency virus/aids

Although many viruses cause suppression of the immune system, only one wipes it out completely, and that is the previously mentioned HIV. It is worth discussing the biology of this virus, which can lead to the well-known AIDS, so that its full effects on the immune system can be understood. The virus is transmitted through semen, vaginal fluids, and blood, and can be caught by risky sexual behaviors and the sharing of needles by intravenous drug users. There are sometimes, but not always, flu-like symptoms in the first 1 to 2 weeks after infection. This is later followed by seroconversion. The anti-HIV antibodies formed during seroconversion are the basis for most initial HIV screening done in the United States. Because seroconversion takes different lengths of time in different individuals, multiple AIDS tests are given months apart to confirm or eliminate the possibility of infection.

Questions & Answers

what is anatomy
Oyindarmola Reply
Anatomy is the identification and description of the structures of living things
Kamara
what's the difference between anatomy and physiology
Oyerinde Reply
Anatomy is the study of the structure of the body, while physiology is the study of the function of the body. Anatomy looks at the body's organs and systems, while physiology looks at how those organs and systems work together to keep the body functioning.
AI-Robot
what is enzymes all about?
Mohammed Reply
Enzymes are proteins that help speed up chemical reactions in our bodies. Enzymes are essential for digestion, liver function and much more. Too much or too little of a certain enzyme can cause health problems
Kamara
yes
Prince
how does the stomach protect itself from the damaging effects of HCl
Wulku Reply
little girl okay how does the stomach protect itself from the damaging effect of HCL
Wulku
it is because of the enzyme that the stomach produce that help the stomach from the damaging effect of HCL
Kamara
function of digestive system
Ali Reply
function of digestive
Ali
the diagram of the lungs
Adaeze Reply
what is the normal body temperature
Diya Reply
37 degrees selcius
Xolo
37°c
Stephanie
please why 37 degree selcius normal temperature
Mark
36.5
Simon
37°c
Iyogho
the normal temperature is 37°c or 98.6 °Fahrenheit is important for maintaining the homeostasis in the body the body regular this temperature through the process called thermoregulation which involves brain skin muscle and other organ working together to maintain stable internal temperature
Stephanie
37A c
Wulku
what is anaemia
Diya Reply
anaemia is the decrease in RBC count hemoglobin count and PVC count
Eniola
what is the pH of the vagina
Diya Reply
how does Lysin attack pathogens
Diya
acid
Mary
I information on anatomy position and digestive system and there enzyme
Elisha Reply
anatomy of the female external genitalia
Muhammad Reply
Organ Systems Of The Human Body (Continued) Organ Systems Of The Human Body (Continued)
Theophilus Reply
what's lochia albra
Kizito
what are the layers of the skin
Helen Reply
It is made up of three layers, the epidermis, dermis, and the hypodermis, all three of which vary significantly in their anatomy and function. The skin's structure is made up of an intricate network which serves as the body's initial barrier against pathogens, UV light, and chemicals, and mechanical
Omer
what is diabetes?
Ifeoluwa
Diabetes is a chronic (long-lasting) health condition that affects how your body turns food into energy. Your body breaks down most of the food you eat into sugar (glucose) and releases it into your bloodstream. When your blood sugar goes up, it signals your pancreas to release insulin. Insulin act
Omer
what is gastric lavage and their implications
Ifeoluwa
what is velium
chizzy
what is a purpose
chizzy
what's fibroid
Kizito
what are disorders of connective tissue
Ester Reply
Rheumatoid arthritis (RA) Scleroderma. Granulomatosis with polyangiitis (GPA) Churg-Strauss syndrome. Lupus. Microscopic polyangiitis. Polymyositis/dermatomyositis. Marfan syndrome.
Omer
arthritis vasculitis
Enitan
what is cardiac output
Okoye Reply
(CO) amount of blood pumped by each ventricle during one minute; equals HR multiplied by SV
AI-Robot
what is SV and HR stand for
David
SV- Stroke Volume HR- Heart Rate
Ebelechukwu
Cardiac output is the amount of blood pumped by the heart in one minute. It's calculated by multiplying the heart rate (the number of times the heart beats in one minute) by the stroke volume (the amount of blood pumped out by the heart with each beat). So, cardiac output = heart rate x stroke volum
Dickson

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Source:  OpenStax, Anatomy & Physiology. OpenStax CNX. Feb 04, 2016 Download for free at http://legacy.cnx.org/content/col11496/1.8
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