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Immunology Exam 3, Part 2
Overview of immunologic disease; Autoimmunity; Alloimmunity
35
Immunology
Professional
03/08/2012

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Term
Define immunodeficiency disorders
Definition
The congenital or acquired absence of one or more components of the immune system
Term
Explain how cancer often/always involves an immunologic abnormality
Definition
1. Immune system is supposed to prevent neoplastic diease

2. Cancer, especially that of the immune system, may result in an immunocompromised host
Term
Define allergic disease
Definition
An untoward immunological response to a foreign immunogen which is called an allergen.

Since the immune system is supposed to respond to foreign immunogens it may be argued in some cases that there is no immunological abnormality involved.
Term
Define alloimmune disease
Definition
An untoward immunological response to a foreign immunogen derived from another member of the same species. It can be correctly argued that this is just a special case of allergy.

But alloimmune disease of newborn, blood transfusion, and tissue transplantation are specialized enough to merit separate consideration.
Term
Describe an ideal immune response
Definition
1. Directed only against immunogens capable of harming the host.

2. Completely effective in totally eradicating the offending immunogen.

3. Completely protective, with no tissue damage from either the immunogen nor the response.
Term
When does tolerance develop?
Definition
Tolerance develops most readily during fetal life and requires the continued presence of the immunogen to remain.

It is more difficult to establish tolerance in adult animals but it can be induced under certain experimental conditions.
Term
What mechanisms allow central tolerance?
Definition
1. Immunogen-induced apoptosis of immature, double-positive T cells

2. Immunogen-induced apoptosis of immature, IgM-expressing B cells
Term
What mechanisms allow peripheral tolerance?
Definition
1. Immunogen-induced anergy of T cells in absence of costimulation

2.Immunogen-induced anergy of B cells in absence of costimulation (T cell help) (possibly)
Term
What mechanisms allow tolerance in mature, immunogen-reactive cells?
Definition
1. Oral tolerance to proteins (Suppressor T cells, anergy)

2. High dose tolerance to aqueous protein administered systemically

3. Low dose tolerance by repetitive doses of immunogens in low concentration
Term
Define autoimmunity
Definition
Autoimmunity is an immune response to self antigens (autoantigens). Since tolerance to autoantigens is considered normal, autoimmunity is considered to be loss of tolerance.
Term
Is autoimmunity the same as autoimmune disease?
Definition
Autoimmunity is not synonymous with autoimmune disease; some autoimmunity is observed in normal individuals.
Term
Define autoimmune disease
Definition
Autoimmune diseases involves tissue damage resulting from immune responses directed against self antigens but this autoimmunity is likely to be the result rather than the cause of the disease.

Essentially all autoimmune disease is idiopathic (spontaneous/from an unknown source)
Term
List five general characteristics of autoimmune disease
Definition
1. Idiopathic
2. Multiple factors (genetics, sex, age, infection, etc)
3. Usually HLA-associated
4. Multiple immunological mechanisms
5. Inability to avoid antigen/unknown cause
Term
List five possible explanations for autoimmunity
Definition
1. Escape of autoreactive T cells from the thymus
2. Breakdown of peripheral tolerance
3. Nonspecific leukocyte activation (ex. superantigens)
4. Molecular mimicry
5. Abnormalities in lymphocyte interactions
Term
How does the TH1 and TH2 balance affect autoimmunity?
Definition
Inflammatory responses are associated with TH1 cells and it has been speculated that an imbalance of TH1 and TH2 cells (which mutually inhibit one another) may contribute to autoimmunity
Term
Describe autoantibodies
Definition
They inhibit (or ocassionally stimulate) important biological receptors, leading to autoimmune disease
Term
List some examples of diseases involving anti-receptor antibodies
Definition
Myasthenia Gravis
Grave's Disease
Insulin-resistant Diabetes
Rhinitis/Asthma
Pernicious Anemia
Term
What are the two classifications an anti-receptor antibody autoimmune disease can fall under?
Definition
Coombs and Gell Type II reactions
Term
What T-cell mediates autoimmunity?

How did scientists discover this?
Definition
Th17 may be the primary mediator cells of autoimmunity, not Th1 as previously thought

IFN-γ deficient mice still had autoimmune disease
Term
Define alloimmunity
Definition
Alloimmunity is an immune response to antigens from individuals of the same species— alloantigens— and is a reflection of the genetic and resulting chemical differences between individuals.
Term
Give two synonyms of alloimmunity
Definition
isoimmunity and isoantigens
Term
List the three main alloantigen systems
Definition
1. ABO blood groups
2. Rh blood types
3. HLA-complex antigens
Term
Describe the minor alloantigen systems
Definition
There are about 600 known erythrocyte antigens and 195 of these are included in the 23 minor blood group systems.
Term
Which alloantigen system is most important for transfusion reactions?
Definition
ABO blood groups
Term
Which alloantigen system is most important for newborn disease?
Definition
Rh blood types
Term
Which alloantigen system is most important for allograft rejection?
Definition
HLA-Complex antigens
Term
What happens during a Rh- mother's first pregnancy with a Rh+ fetus?
Definition
Minor destruction of fetal erythrocytes by anti-Rh IgG, but healthy newborn
Term
What happens during a Rh- mother's second and subsequent pregnancy with a Rh+ fetus?
Definition
Major destruction of fetal erythrocytes by anti-Rh IgG, anemic newborn

Unless treated with RHO (D) IMMUNE GLOBULIN [HUMAN]
Term
What happens during a Rh- mother's second and subsequent pregnancy with a Rh+ fetus if she is treated with RHO (D) IMMUNE GLOBULIN?
Definition
B cells think they are seeing self and shut off (inducing tolerance), primary immune response inhibited, healthy baby
Term
What increases medical need for transfusion?

What decreases medical need for transfusion?
Definition
More need: cancer chemotherapy

Less need: biologics like epoetin and oprelvekin
Term
In a medical emergency, if a patient has never received a transfusion before could you give him any blood type?
Definition
Yes, because there is a lack of memory. Multiple transfused patients more likely to exhibit transfusion reactions.
Term
What drugs can be given to manage a transplantation reaction?
Definition
Cyclosporine and other immunosuppressive agents
Term
Define Graft-Versus-Host Disease
Definition
A potential problem whenever immunologically-competent tissue is grafted into an immunologically-deficient patient. The most common clinical situation is bone marrow transplantation.
Term
What is the mechanism of action of Cyclosporine A?
Definition
Cyclosporine A inhibits production of IL-2
Term
What is the difference between hyper acute and acute transplantation reaction?
Definition
Hyper acute is very fast due to pre-existing antibodies

Acute is T-cell mediated
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