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Medical Pathology and Genetics
PPT4 Diseases of Immune System
73
Other
Graduate
01/27/2012

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Cards

Term
Name and describe the three levels of defense of the body.
Definition
1. The skin and organisms that inhabit it: Skin is a barrier; organisms set up unfavorable conditions for pathogens

2. Nonspecific Reaction- Inflammation: blocks entry of pathogens into the body, blocks spread of pathogens if they enter the body, eliminates pathogens and damaged cells, activation of the adaptive immune system

3. **Specific response: immune response
Term
In the complement system, what does the C3 convertase do?
Definition
Converts C3 to C3a and C3b
Term
What are stem cells?
Definition
cells in bone marrow that produce lymphocytes by mitotic division
Term
What are lymphocytes?
Definition
WBCs that originate in bone marrow and mediate the immune response: B cells, T cells
Term
What are the two main lymphocytes in the immune system?
Definition
B cells and T cells
Term
What are B cells and what do they do?
Definition
They are a type of lymphocyte that remains in the bone marrow until it matures

They produce antibodies

They control antibody-mediated (humeral) immunity
Term
What is a T cell and what does it do?
Definition
A type of lymphocyte that migrates to the thymus gland to mature

Has T cell receptors that bind to infected cells

Controls cell-mediated (cellular) immunity
Term
The immune system has two interconnected parts, what are they and what connects the two?
Definition
-Antibody-mediated immunity, regulated by B cells and antibody production
-Cell-mediated immunity, controlled by T cells

The two systems are connected by helper T cells
Term
B cells are genetically programmed to produce large quantities of unique ____________
Definition
antibodies
Term
What are antibodies?
Definition
Proteins produced by B cells that bind to specific foreign molecules (antigens) and inactivate them
*some circulate in the blood and lymph; others remain attached to the surface of B cells
Term
What are antigens?
Definition
Molecules that initiate antibody production, carried or produced by microbes
Term
Antibodies are Y-shaped protein molecules secreted by _______ (_______) __/________ that bind to specific antigens to form antigen-antibody complex
Definition
effector (plasma) B cells
Term
Antibodies belong to a large family of proteins known as __________ molecules. Define these molecules.
Definition
-Immunoglobulin (Ig)
-five classes or isotopes of proteins to which antibodies belong (IgM, IgG, IgA, IgE, IgD), each with unique structure, size, and function
Term
Describe IgM's
Definition
-Found on the surface of B cells and plasma cells
-very effective at killing bacteria
-avid complement activator
**First immunoglobulin to appear after immunization
-Natural antibody against blood group antigens A and B
Term
Describe IgG's
Definition
-MOST ABUNDANT immunoglobulin in blood plasma
-produced during primary and secondary response
-**passes through placenta, providing protection to the fetus
-coats microbes, facilitating their phagocytosis
Term
Descibe IgA's
Definition
-produced by plasma cells in digestive, reparatory and urinary system
-Concentrates in body fluids and guards entrance to the body**
Term
Describe IgE's
Definition
-present in trace amounts in serum
-produced by plasma cells in skin, tonsils, and digestive and respiratory systems
-coats and kills helminthic parasites**
-attaches to mast cells and mediates allergic reactions**
Term
Describes IgD's
Definition
remains attached to B cells and plays a role in initiating early B cell response
Term
What is the structure of an antibody?
Definition
antibodies are composed of two identical heavy-chain (H) polypeptides and two identical light-chain (L) polypeptides
Term
How are antibodies formed?
Definition
Somatic recombination in the heavy-chain (H) locus and in two light chain (L) loci during B cell maturation form billions of unique antigen-binding sites (structure is related to function)
Term
What are 2 functions of antibodies?
Definition
-to recognize and bind antigens
-to inactivate antigens
Term
Describe how antibodies bind to specific antigens
Definition
in a "lock and key" manner
Term
What are the regions on the light chains and heavy chains of an antibody where recombination occurs?
Definition
-Heavy chain= V-D-J region
-Light chain= V-J region
Term
What are T-cell receptors (TCRs)?
Definition
unique proteins on the surface of T cells that bind to specific proteins on the surface of cells infected with viruses, bacteria, or intracellular parasites
*T-cells are programmed in the thymus to produce unique T cell receptors
Term
Each B cell makes only one type of _________ and each T cell makes only one type of _____________.
Definition
antibody
receptor
Term
Binding of an antibody on a B cell to an antigen stimulates what to happen?
Definition
stimulates the B cell to divide, producing many B-cell clones with the same antibody
Term
Binding of a TCR on a T cell to an antigen stimulates what to happen?
Definition
stimulates that T cell to divide, producing many T-cell clones with the same TCR
Term
What is the major histocompatability complex (MHC)?
Definition
-genes on chromosome 6 that encode cell recognition molecules that prevent the immune system from attacking a body's own organs and tissues
-Antigens attach to MHC molecules on surface of cell in order to activate cell mediated immunity
Term
Define antibody-mediated immunity.
Definition
-immune reaction mediated by B cells that protects against invading bacteria and viruses before they infect a cell using antibodies produced by effector (plasma) B cells
Term
Define cell mediated immunity
Definition
Immune reaction mediated by T cells directed against body cells that have been infected by viruses, parasites, some bacteria, cancer cells.
Term
What are 2 functions of a helper T cell?
Definition
-stimulates production of antibodies by B cells when an antigen is present
-stimulates division of B cells and cytotoxic T cells
Term
What is the process of the antigen detection stage of antibody-mediated immune response
Definition
Naive B cell antibody binds to antigen and becomes antigen-presenting B cell
Term
What is the process of the activation of helper T cell stage of antibody-mediated immune response
Definition
Naive Helper T cell TCR binds to antigen-MHC on antigen presenting B cell, which activates the helper T cell
-activated helper T cell activates the B cell
Term
What is the process of antibody production by B cell during antibody mediated immune response?
Definition
activated B cell forms two types of daughter cells: effector B cells and memory B cells
Term
What are effector B cells?
Definition
daughter cells of B cells, which synthesize and secrete 2,000 to 20,000 antibody molecules per second into the bloodstream
Term
What are memory B cells?
Definition
a long-lived B cell produced after primary exposure to an antigen that plays an important role in secondary immunity
Term
What is a big physical difference between unactivated and activated B cells?
Definition
activated has more RER where proteins (antibodies) are being made
Term
What is the function of Helper T cells (CD4+)
Definition
stimulate division of B cells, cytotoxic T cells, and production of antibodies by B cells
Term
What is the function of suppressor T cells (CD8+)
Definition
stop immune responses of B cells, other T cells
Term
What is the function of cytotoxic "killer" T cells (CD8+)
Definition
Secrete perforin to destroy infected body cells; directly attack viruses, bacteria, cancer cells, and transplanted organs
Term
What is the function of memory T cells?
Definition
activate the immune response if the same antigen is reintroduced
Term
What is a primary immune response?
Definition
B and T memory cells are produced as a result of the first infection by a specific antigen
Term
What is a secondary immune response?
Definition
a second exposure to the same antigen (as in the primary immune response) results in an immediate, large scale production of killer T cells and antibodies
-the second response is faster, larger, and longer lasting than the first
Term
What is a vaccine?
Definition
a preparation containing dead or weakened pathogens that elicits an immune response when injected into the body that produces memory cells
Term
In ABO blood typing, individuals produce antibodies against any cell surface antigens that they do OR do not have??
Definition
they produce antibodies against any cell surface antigens they do NOT have
Ex; A blood type= has A antigen on the surface of the RBCs
Term
What is Hemolytic disease of the newborn (HDN)?
Definition
a condition of immunological incompatibility between the mother and fetus that occurs when the mother is Rh- and the fetus is Rh+
*Rh+ express Rh D antigen on their blood cells
*Rh- do NOT express Rh D antigen
Term
What happens if a women who is Rh- gives birth to a baby who is Rh+?
Definition
The mother's body will produce antibodies against the Rh+ cells. If she gets pregnant again and the baby is Rh+, the mother's circulating Rh+ antibodies will act against it
Term
What is a haplotype? What is a good example of a haplotype?
Definition
a cluster of closely linked genes or markers that are inherited together
-HLA alleles on a specific copy of chromosome ^ are a haplotype
Term
What is graft-versus-host disease?
Definition
T cells from allogenic donor bone marrow or tissue react against recipient's antigens
Term
What are hypersensitivity diseases?
Definition
disorders caused by excessive or abnormal activation of the immune system in response to exogenous antigens or endogenous autoantigens
Term
Hypersensitivity diseases are pathologically classified into four major groups, what are they?
Definition
-Type I: immediate reaction
-Type 2: antibody mediated reaction
-Type 3: immune complex-mediated reaction
-Type 4: T-cell mediated reaction
Term
What is an example of a Type 1 hypersensitivity?
Definition
-allergic reactions
-allergies are the result of immunological hypersensitivity to weak antigens that do not provoke an immune response in most people
Term
What are allergens?
Definition
-weak antigens that provoke an inappropriate immune response
-Include house dust, pollen, cat hair, certain foods, and medicine such as penicillin
Term
What are some clinical examples of type 1 hypersensitivity?
Definition
-hay fever, atopic dermatitis, bronchial asthma, anaphylactic shock
Term
What are the steps of a type 1 hypersensitivity response? (5)
Definition
-environmental antigen binds to antibody on naive B cell
-activates helper T cells
-B cells produce IgE
-IgE attaches to mast cells
-mast cells release mediators
Term
Type 2 hypersensitivity reaction is mediated by which immunoglobulins?
Definition
IgG or IgM
Term
What are 4 clinical examples of Type 2 mediated hypersensitivity?
Definition
-autoimmune hemolytic anemia
-goodpasture syndrome
-myasthenia gravis
-graves disease
Term
Describe type 3 hypersensitivity
Definition
a reaction mediated by immune complexes (antigens-antibody) that are formed in the circulation, deposit in vascular beds, stimulating complement activation and acute inflammation
Term
What is the major clinical example of type 3 hypersensitivity?
Definition
SLE
Term
Describe type IV hypersensitivity reaction
Definition
(delayed type hypersensitivty or T-cell mediated cytotoxicity) is mediated by T lymphocytes and macrophages, which typically aggregate at the site of injury to form granulomas
Term
What are some major examples of type 4 hypersensitivity?
Definition
-type 1 diabetes
-MS
-rheumatoid arthritis
-Crohn disease
-contact dermatitis
Term
What is self tolerance?
Definition
the lack of immune responsiveness to one's own tissue antigens
Term
What is central tolerance?
Definition
immature lymphocytes that recognize self antigens in the central lymphoid organs (B cells in the bone and T cells in the thymus) are killed by apoptosis
Term
What is peripheral tolerance?
Definition
mature lymphocytes that recognize self-antigens in peripheral tissues are: functionally inactivated, suppressed by regulatory lymphocytes, die by apoptosis
Term
Describe SLE
Definition
systemic autoimmune disease caused by autoantibodies against self antigens (mainly nuclear antigens) and formation of immune complexes
-a chronic inflammatory CT disease that affects many organs, highly variable symptoms
-inherited susceptibility in Class 2 MHC and complement genes
Term
Describe rheumatoid arthritis
Definition
Chronic, systemic, inflammatory autoimmune disease that affects mainly the joints
-proposed environmental trigger is activation of helper T cells by self antigen or microbial antigen
Term
Describe HIV
Definition
-a secondary immune deficiency
-HIV is a retrovirus that selectively infects and kills the T helper cells
-it is a virus in RNA that injects itself into your helper T cell that have a reverse transcriptase and turns the viral RNA into viral DNA which will go insert into the genome
Term
Describe the acute phase of HIV infection
Definition
-infection of memory T cells in mucosal lymphoid tissues
-high levels of virus production: viremia
-signs of systemic infection
Term
Describe the chronic phase of HIV infection
Definition
-virus is continuing to replicate
-immune system still largely intact
-minor opportunistic infections
-immune defenses diminish
-may last 7-10 years
Term
Describe the crisis phase of HIV
Definition
-catastrophic breakdown of immune defenses
-marked increase in viremia
-AIDS defining conditions (serious opportunistic infections, secondary neoplasms, neurologic manifestations)
Term
What is amyloidosis?
Definition
a disorder characterized by the extracellular deposits of misfiled proteins that aggregate to form insoluble fibrils called amyloid
Term
Amyloidosis is defined based on the physical properties of the fibers, what are they (3)?
Definition
-7.5-10 nm in diameter
-arranged in beta pleated sheets
-identified by Congo red staining
Term
Definitive diagnosis of amyloidosis can only be determined by what?
Definition
biopsy (demonstrating amyloid in tissue)
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