Term
|
Definition
| exposure to an antigen elicts a detectable reaction |
|
|
Term
| define hypersensitivity reaction |
|
Definition
antigen specific harmful immune response after a primining or sensitization encounter
produces tissue injury and dysfunction |
|
|
Term
| how are allergies acquired |
|
Definition
| genetic predisposition on HLA gene |
|
|
Term
|
Definition
| give rise to immediate hypersensitivity |
|
|
Term
| what are some common allergens |
|
Definition
| foreign serum, vaccines, ragweed, rye grass, timothy grass, birch tree, penicillin, sulfonamides, anestheics, salicylates, nuts, seafood, egg, pears, beans, milk, bees, wasps, ants, cockroach calys, dust m ites, mold spores, animal hair, animal dander |
|
|
Term
|
Definition
| genetic predisposition to make inappropirate levels of IgE specific to allergens |
|
|
Term
| what are the four types of hypersensitivity, briefly describe them |
|
Definition
1. immediate or anaphylactic
2. antibody mediated
3. immune complex
4. delayed (T cell) mediated |
|
|
Term
| how long does it take hypersesnsitivy 1 to appear |
|
Definition
|
|
Term
| what are the 5 processes in type one hypersensitivity |
|
Definition
induction of plasma cells
degranulation of mast cells
anaphylactic shock
phagocyte independent eosinophil mediated immunity |
|
|
Term
| how are plasma cells induces, what type of hypersensitivity is this |
|
Definition
type 1
Th2 sees APC with microbe and releases IL-4 which tells B cell to turn into a plasma cell and make IgE via class switch from IgM
some people have a genetic predisposition to this |
|
|
Term
| what is the process of mast cell degranulation |
|
Definition
| antigen combines with IgE on mast cells, IgE binds to FceRI on mast cells and basophils (sensitizes). antigen bridges gap between two antibodies causing degranulation |
|
|
Term
| what processes are begun or mediators released upon mast cell degranulation (5) |
|
Definition
histamine, prostaglandins, leukotrienes, cytokines
mast cells aid in response to helminths |
|
|
Term
| what is the main dangerous process in anaphylactic shock |
|
Definition
| massive drop in blood pressure that can be fatal in minutes |
|
|
Term
| how do eosinophils contribute to type 1 hypersensitivity, how are they stimulated to act |
|
Definition
effective against helminths
stimulated by TH2 via IL-5 |
|
|
Term
| what are the five clinical reactions that can occur due to type 1 hypersensitivity |
|
Definition
allergic rhinitis
food allergies
bronchial asthma
systemic anaphylaxis
local anaphylaxis |
|
|
Term
| what are three clinical signs of allergic rhinitis |
|
Definition
| mucous, inflammation of upper airway, sinuses full |
|
|
Term
| what are two clinical signs of food allergy |
|
Definition
| increased peristalsis, intestinal contraction |
|
|
Term
| what are three clinical signs of bronchial asthma |
|
Definition
| bronchial smooth muscle contraction, inflammation, tissue injury (late phase) |
|
|
Term
| what allergins commonly cause systemic anaphylaxis, why is this scary |
|
Definition
food, drug, bee sting, etc
can be fatal in minutes |
|
|
Term
| what are the three components/parts of the body that respond in anaphylaxis |
|
Definition
| vascular, respiratory, GI |
|
|
Term
| what is the vascular response to systemic anaphylaxis (5) |
|
Definition
vasodilation drops BP (hypotension)
swelling of tissues (edema) especially tongue
reduced oxygen to tissues
irregular heart beat
shock and loss of conciousness |
|
|
Term
| what is the respiratory response to anaphylaxis (5) |
|
Definition
airway obstruction due to laryngeal edema
smooth muscle contraction causes bronchial obstruction
difficulty swallowing
difficulty breathing and wheeziing |
|
|
Term
| what is the GI response to systemic anaphylaxis (3) |
|
Definition
contraction of smooth muscle causes cramps and vomiting
fluid outflow into the gut causes diarrhea |
|
|
Term
| what is local anaphylaxis, what three systems does this commonly occur in, explaiin some signs in each |
|
Definition
target organ response to allergin
digestive tract: cramping, diarrhea, vomiting
skin: redness, inflammation, itching
airway: sneezing, rhinitis OR wheezing, asthma |
|
|
Term
| what 4 drugs are commonly used to treat type 1 hypersensitivity |
|
Definition
non-steroid anti-inflammatory
antihistamines block histamine receptors
steroids
throphylline/epinepherine |
|
|
Term
| how does throphylline/epinepherine work |
|
Definition
| increaes cAMP in mast cells inhibiting degranulation |
|
|
Term
| define hyposensitization, what is a clinical example of this |
|
Definition
immunotherapy/allergy shots
desensitizing the immune system. repeated injections of the allergin reduces IgE and prodces IgG instead |
|
|
Term
| type 2 hypersensititivy: how does it work |
|
Definition
| anto-antibodies (IgM or IgG) bind to cells and cause lysis or phagocytosis |
|
|
Term
| how does IgM or IgG cause lysis or phagocytosis in type 2 hypersensitivity (4) |
|
Definition
binds antigen on target for phagocytosis
complement and Fc receptor mediated inflammation and tissue injury
interference of normal cellular function by binding to physiologically important molecules of cellular receptors
antibodies inhibit binding of neurotransmitters |
|
|
Term
| what are 11 diseases or conditions that display type two hypersensitivity |
|
Definition
grave's disease
myasthenia gravis
idiopathic theombytopenic purpura (ITP)
phemphigus vulgaris
goodpasture-s syndrome
acute rheumatic dever
pernicious anemia
cytotoxic reactions
transfusion reaction
hemolytic disease of the newborn
drug reactions |
|
|
Term
| what type of hypersensitivity is a drug reaction |
|
Definition
|
|
Term
| graves disease: aka, disease process, 2 clinical symptoms, type of hypersensitivity |
|
Definition
type 2 hypersensitivity - autoimmune
hyperthyroidism
antibody attaches to TSH receptor on thyroid epithelial cell causing thyroid hormone production without TSH
causes goiter (enlarged thryoid) and bulging eyes |
|
|
Term
| myasthenia gravis: disease process, 3 clinical symptoms, treatment, type of hypersensitivity |
|
Definition
type 2 hypersensitivity - autoimmune
B- cell disease
auto-antibody attaches to ACh receptor and inhibits binding of neurotransmitter
causes progressive muscle weakness and paralysis (mostly in women), ptosis
survival is dependent on immune supressants |
|
|
Term
| idipoathic themobyctpenic purpura: process, cause, what other disease is it associated with, what is the main symoptom, type of hypersensitivity |
|
Definition
type 2 hypersensitivity
antibody to platelets causes opsonization and phagocytosis
mostly in women after bacteria or viral infections
associated with autoimmune systemic luus (SLE)
bleeding problems |
|
|
Term
| phemphigus vulgaris: disease process, symptoms, type of hypersensitivity |
|
Definition
type 2 hypersensitivity
epidermal cadherin proteins between epidermal cells targetd by antibodies
proteases are activated and disrupt adhesions
skil vesicles (bullae) |
|
|
Term
| goodpastures syndrome: disease process, 2 clinical signs, type of hypersensitivity |
|
Definition
type 2 hypersensitivity
protein in basement membrane of the kidney glomeruli and lung alveoli is targeted by antibodies
causes complement and Fc receptor mediatedi inflammation
leads to nephritis and lung hemorrhages |
|
|
Term
| acute rheumatic fever: disease process, 2 clinical signs, type of hypersensitivity |
|
Definition
type 2 hypersensitivity
strep cell wall antigen cross reacts with myocardial antigen
causes inflammation and macrophage activation
myocarditis, arthritis |
|
|
Term
| pernucuius anemia; disease process, 2 clinical signs, type of hypersensitivity |
|
Definition
type 2 hypersensitivity
intrinsic factor of gastric parietal cells is targeted by antibodies decreasing B12 absorption
erythropoisesis, anemia |
|
|
Term
| what are the three parts of a cytotoxic reaction, what type of hypersensitivity is this |
|
Definition
type 2 hypersensitivity
IgG or IgM actiate complement when they bind to an antigenic cell and the cell is lysed
C5a and C3a activate inflammation and tissue injury via neutrophils, enzymes, ROS
C3b opsonizes cells and allows for phagocytosis |
|
|
Term
| transfusion reactions: what two areas can cause issues, explain why, who are two associated diseases |
|
Definition
ABO blood group: O is universal donor, incompatable donor cells are lysed as they enter the blood
RH reactions: hemolytic disease of the newbown, autoimmune hemolytic anemia |
|
|
Term
| what occurs in hemolytic disease of the newborn, what type of hypersensitiviy this it |
|
Definition
type 2 hypersensitivity
fetal cells are destoried by maternal anti0Rh antibodies that cross placenta |
|
|
Term
| what occurs in autoimmune hemolytic anemia, what are 2 clinical symptoms, what type of hypersesnitivity is this |
|
Definition
type 2 hypersensitivity
Rh antigens lead to opsonization by phagocytosis
causes hemolysis and anemia |
|
|
Term
| what are the clinical signs of a type 2 hypersensitivity drug reaction |
|
Definition
| hemolytic anemia, thrombocytopenia |
|
|
Term
| type 3 hypersensitivity: how does it work |
|
Definition
IgG complexes with antigen (self oor foreign) and attaches to tissue and they are overproduced or not cleared
complex activates complement which attracts neutrophils which release lysosomal enzymes and cause damage |
|
|
Term
| what areas do type three hypersensitivity complexies deposit on |
|
Definition
| vessel wall, synovial membranes of joints, glomerular membranes of the kidney, choroid plexus of the brain |
|
|
Term
| type 3 hypersensitivity complexes are deposited in perdictable places based on what |
|
Definition
| the introduction method of the antigen |
|
|
Term
| when the antigen is introduced via IV where might it deposit and what clinical symptoms does that cause (3) |
|
Definition
IV:
vessel wall > vasculitis
renal glomeruli > nephritis
joint space > arthritis |
|
|
Term
| when the antigen is introduced via subcutaneous where might it deposit and what clinical symptoms does that cause (2) |
|
Definition
| perivascular space > arthys reaction |
|
|
Term
| when the antigen is introduced via inhalation where might it deposit and what clinical symptoms does that cause (1) |
|
Definition
| attach to lung (farmer's lung) > alveolar or capillary interference |
|
|
Term
| what seven diseases are associated with type 3 hypersensitivity |
|
Definition
arthritis
glomerulonephritis
systemic lupus erythematosus
rheumatoid arthritis
polarteritis nodosa
serum sickness
arthus reaction |
|
|
Term
| arthritis: MOA, type of hypersensitivity |
|
Definition
type 3 hypersensitivity
complexes deposit in joints and cause local inflammation |
|
|
Term
| glomerulonephritis: cause |
|
Definition
post strep infection
complexes deposit in kidney causing nephritis |
|
|
Term
| systemic lupus erythematosus: 4 causes, hypersensitivity type |
|
Definition
type 3 hypersensitivity
estrogen stimulates B cells to make IL-10 and produce autoantibodies reaction to DNA, blood cells, neurons, and tissues
lack of T cells
activation of complement
cells die and immune complexes form and deposit under skin, joints, kidney, vessels, and cns |
|
|
Term
| systemic lupus erythematosus: most important symptoms (6) |
|
Definition
bitterfly rash on fase
nephritis, arthritis
vasculitis
inflammation interferes with function of the sites
kidney damage (lupus nephritis) - most common cause of death |
|
|
Term
|
Definition
|
|
Term
| who does rheumatoid arthritis effect |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| rheumatoid arthritis: how does it work, what type of hypersensitivity |
|
Definition
type 3 hypersensitivity
may involve molecular mimicry
IgM antibodies (Rh factors) against IgG form complexes in joints causing inflammation and catiladge damage causing joint deformities (finger) |
|
|
Term
| what are the five parts of RA treatment |
|
Definition
anti-inflammatory (asprin)
immune supressants
physical therapy to keep joints moving
surgical replacement of joints |
|
|
Term
| polarteritis nodosa: cause, clinical issue, type of hypersesntitivyt |
|
Definition
type 3 hypersensitivity
HebB virus surface antigen causes complexes leading to vasculitis |
|
|
Term
| serum sickness: cause, 3 clinical changes, type of hypersensitivity |
|
Definition
type 3 hypersensitivity
various protein antigens cause complexes that lead to systemic vasculitis, nephritis, and arthritis |
|
|
Term
| arthus reaction: cause, clinical change, type of hypersensitivity |
|
Definition
type 3 hypersensitivity
various protein antigens cause complexes that lead to coutaneous vasculitis |
|
|
Term
| type 4 hyersensitivity cause |
|
Definition
| sensitized T cell encounter antigen and release cytokines that activate macrophages and cause local inflammation |
|
|
Term
| how does CD4 activate macrophages |
|
Definition
|
|
Term
| how long does it take for a type 4 hypersensitivity to appear, why |
|
Definition
takes time for macrophages to be activated and micrage to the site
1-2 days |
|
|
Term
| what are symptoms of type 4 hypersensitivity (5) |
|
Definition
| itching, redness, swelling, paini, anaphylactic shock |
|
|
Term
| what are examples of type 4 hypersensitivity (6) |
|
Definition
| TB skin test, poison ivy, metal reaction, latex reaction, contact dermatitis, insulin dependant (type 1) diabetes mellitus |
|
|
Term
| contact dermatitis: proces, type of hypersensitivity |
|
Definition
type 4 hypersensitivity
small molecules act as haptens and complex with skin proteins
they are tkane by APC to TH1 and sensitize the T cell
during second exposure TH1 memory sell is activated and causes dermatitis |
|
|
Term
| what are the symptoms of contact dermatitis (5) |
|
Definition
| granulomas, chronic infection, itching, redness, uricaria |
|
|
Term
|
Definition
multinucleated giant cell surounded by epitheloid cells with intracellular bacteria
surrounded by macrophages and TH1 sensitized to the antigen |
|
|
Term
| insulin dependent diametes mellitus: cause, type of hypersensitivity |
|
Definition
type 4 hypersensitivity
insuffieient insulin production due to immunological destruction of insulin secreting cells of the pancreas |
|
|
Term
| what are the complications of insulin dependent diabetes mellutis (5) |
|
Definition
gangrene, kidney and cardiovascular disease
dehydration
nerve damage |
|
|
Term
| TB: MOA, type of hypersensitivity |
|
Definition
type 4 hypersensitivity
TH1 and macrophage make caseous regions and cause tissue destruction
granuloma / tubercule formation |
|
|
Term
| PPD, what is it made of, how is it given, when is it read, what indicates positive, what type of hypersensitivty |
|
Definition
type 4 hypersensitivity
protein from mycobaterial cell wall
intradermal injection
read in 48-72 hours for red, swollen reaction |
|
|
Term
| what is an immune deficiency - basic |
|
Definition
|
|
Term
| define hypersensitivity - basic |
|
Definition
|
|
Term
| define autoimmunity - basic |
|
Definition
misdirected immune system
immunity to self
immune system attacks and destorys healthy body tissue |
|
|
Term
| in what two areas could there be an issue with autoimmine disorders |
|
Definition
innate immunity (complement)
acquired immunity - cell mediated (T cells) and humoral (B cells) |
|
|
Term
| who gets autoimmunity more often, why |
|
Definition
| women due to more hormones and higher stress |
|
|
Term
| what are the three causes of autoimmunity |
|
Definition
genetic predisposition
enivornment
immune regulation |
|
|
Term
| how does genetics cause autoimmunity |
|
Definition
hormone levels
gene mutations (not one gene) |
|
|
Term
| how can the enivrionment cause autoimmunity (5) |
|
Definition
virus, bacteria, drug (lupus), injury
can trigger a genetic component |
|
|
Term
| how does immune regulation cause autoimmunity |
|
Definition
| difficult to tell if T or B cell due to the complexity of the immune system |
|
|
Term
| once autoimmunity is formed by one of the three pathways, what causes the issues in the body, how |
|
Definition
complement system
protective when functioning against pathogens
mediator in pathogenesis and prevention of immune complex disease
inflammation promoted by it can cause cellular damage
can affect any organ system in the body |
|
|
Term
| name 9 organ specific autoimmune diseases |
|
Definition
type 1 diabetes mellitus
goodpasture's syndrome
MS
grave's disease
hashimoto's thyroiditis
autoimmune pernicious anemia
autoimmune addisons disease
vitiligo
myasthemia gravis |
|
|
Term
| name 5 systemic autoimmune disease |
|
Definition
rheumatoid arthritis
scleroderma
systemic lupus erthematosus
primary sjorgen's syndrome
polymyositis |
|
|
Term
| what are three ways infection can lead to autoimmunity |
|
Definition
molecular mimicry
superantigens
binding of pathogen to self protein |
|
|
Term
| what are three examples of microbes that trigger autoimmunity and the associated disease |
|
Definition
group A strep - rheumatic fever
chalmydia - reiter's syndrome
borelia burgdorferi - perdisposition to lyme disease |
|
|
Term
| how does molecular mimicry acuse autoimmunity, what are three disease examples |
|
Definition
production of cross reative antibodies or T cells
rheumatic fever, diabetes, MS |
|
|
Term
| rheumatic fever: cause, disease process, 2 clinical symproms, type of hypersensitivity |
|
Definition
type 3 hypersensitivity
group A strep presents in throat infection and expresses M protein
IgG produced against M protein binds to cardiac cells similar to M protein
injury to heart valves, sarcolemma |
|
|
Term
| what is the normal function of M protein, where is it found |
|
Definition
| virulence on group A strep that helps evade phagocytosis |
|
|
Term
| what is a superantigen, how does it work, what disease does it cause |
|
Definition
immune system sees something foreign and goes crazy
polyclonal activation of autoreactive T cells
causes rheumatoid arthritis |
|
|
Term
| what autoimmune diseae does group A strep cause, what are two clinical signs |
|
Definition
rheumatic fever
carditis, polurathritis |
|
|
Term
| in autoimmunity there is binding of pathogen to self protein, how does this contribute to the disease, what condition can this cause |
|
Definition
pathogen acts as a carrier to allow anti-self response
interstitial nephritis |
|
|
Term
| chalmydia trachomatis: what gene is is associated with, what disease does it cause, what symptom |
|
Definition
HLA B27
reiter syndrome
arthritis |
|
|
Term
| what gene does borrelia burgdorferi trigger, what does this cause clinical, what is the symptom |
|
Definition
associated with HLA DR2 and DR4
leads to predisposition for lyme disease
chronic arthritis |
|
|
Term
| what diseases is HLA B27 associated with (3) |
|
Definition
ankylosing spondylitis
acute anterior uveitis
chalmydia trachomatis |
|
|
Term
| what diseases is HLA DR2 associated with (3) |
|
Definition
| goodpasture's syndrome, MS, Lyme disease |
|
|
Term
| what diseases is HLA DR3 associated with |
|
Definition
|
|
Term
| what disease is HLA DR4 associated with |
|
Definition
|
|
Term
| how does estrogen regulate the immune system (2) |
|
Definition
stimulates prolactin to help regulate immune response,
stimulates corticotropin releasing hormone promoting corticol secretion |
|
|
Term
| how does estrogen trigger autoimmunity |
|
Definition
| causes TH1 dominated immune response promoting inflammation |
|
|
Term
| what are 5 pieces of evidence that show females have a higher chance for autoimmunity |
|
Definition
females producer higher antibody titers
bigger immune response
higher cortisol secretion
higher CD4 levels
higher serum IgM |
|
|
Term
| what is the role of testosterone in immunity |
|
Definition
| causes or protects against autoimmunity |
|
|
Term
| how do sex hormones alter immune response |
|
Definition
| influence gene expression |
|
|
Term
| what is the CC of a female and the common age that you should suspect autoimmunity |
|
Definition
joint pain and fatigue
20s and 30s |
|
|
Term
| what diseases do makes haver a higher chance of autoimmunity in (2) |
|
Definition
ulcerative colitis is equal to women
diabetes mellitus is more male |
|
|
Term
| when does systemic lupus present, why |
|
Definition
| women during the age 15-45 in the height of estrogen production |
|
|
Term
| what is the role of iL-10 in lupus |
|
Definition
| it is commonly seen and is high and it is an activtor of B cells which make autoreactive antibodies to self proteins |
|
|
Term
| what are the two rashes in lupus, how is it caused |
|
Definition
malar rash: butterfiy red rash, raished or flat, worse in sun
discoid rash: stimulated y UV light, raised patch with scaling skin cause by band like deposition of immune complexes at dermal epidermal junctions |
|
|
Term
| list a lot of lupus symptoms ( |
|
Definition
face rashes
arthritis inflammation - hands, wrist, knees, stops normal function (small
joints)
anorexia
malaise
fever
weight loss
oral ulcers: painless
fatigue: common, persists
raynauds phenomenon
lypus nephritis
seizures
nerve paralysis
depression
psychosis
clots in vessels to spinal cord
thrmobocytpenia
pericarditis
myocarditis: looks like viral
libman sacks endocarditis
valvular disease
hypertension
cardiac failure
alopecia - hair loss |
|
|
Term
| what is their vasculitis in lupus |
|
Definition
antigen antibody complexes deposit into vessel wall causing vasculitis and ulceration
accouns for many of the signs and symptoms |
|
|
Term
| what causes fatugue in lupus (3) |
|
Definition
| low grade fever, anemia, inflammation |
|
|
Term
| what is raynaud's phenomenon, what diseases is it seen it |
|
Definition
not specific to lupus but common in it
triphasic reaction of digits to cold or emotion
skin changes from white to blue to red |
|
|
Term
| what causes lupus nephritis |
|
Definition
| glomerular nephritis caused by cellular proliferation |
|
|
Term
| why is there thrombocytopenia in lupus |
|
Definition
| antiplatelet antibodies or antiphospholipid antibodies |
|
|
Term
| what is the most common cardiac manigestation of SLE |
|
Definition
|
|
Term
| libman-sacks endocarditis: what is it, what disease is it seen in |
|
Definition
flat reddish tan vegitations on mitral valve and chordae
seen in SLE |
|
|
Term
| what is the treatment for lupus (2) |
|
Definition
antiinflammatory
immune supressants |
|
|
Term
| what are the cells of the islets of langerhan (3) what do they make |
|
Definition
alpha cells - glucagon
beta cells - insulin
gamma cells - somatostatin |
|
|
Term
| what is the MOA of type 1 diabetes |
|
Definition
effector T cell recognizes peptides from c specific protein and kills the B cell
glucgagona and somatostatin are still made |
|
|
Term
| 5 complications of type 1 diabetes, type of hypersensitivity |
|
Definition
type 4 hypersesnitivity - autoimmunity
gangrene, kidney and cardiovascular disease, dehydration, nerve damage |
|
|
Term
| when does type 1 diabetes normally develop |
|
Definition
|
|
Term
|
Definition
autoantibodies or self reactive t cells cause demyelination of nerves
causes inflammation and scaring |
|
|
Term
| secondary sjogern's syndrome: cause, secondary to what (2), symptoms (2) |
|
Definition
secondary to RA, SLE
dry eyes, dry mouth
exocrine glands infiltrated with lymphocytes |
|
|
Term
| general treatments for autoimmunity (7) |
|
Definition
antiinflammatory: NSAID, corticosteroids
immune supressants: methotrexate
radiation
plampheresis
cell blocking reagents
cytokine blocking reagents (TNG
humira, embrel)
plant sterols? |
|
|
Term
| innate immunity: 5 examples, definition, 2 defects |
|
Definition
non specific 1st line of defense
epithelial barrier, phagocytes, NK cells, blood proteins, cytokines
phagocyte defects in migration or killing, complement defects |
|
|
Term
| adaptive immunity: definition, three categories of defects |
|
Definition
specific or memory immunity
humeral, cell mediated, both |
|
|
Term
| humeral immunity: definition or putpose |
|
Definition
provides defense against extracellular microbes
mediated by antibodies |
|
|
Term
| name three humeral immunity defects |
|
Definition
absence of mature cells, plasma cells, Ig or select Ig
quantative and qualitative |
|
|
Term
| what are the 4 signs of humeral immunity defect |
|
Definition
serium Ig reduced
increased bacterial infections
delayed type hypersensitivity reactions are normal
lymphoid tissue has less germinal centers and follicles |
|
|
Term
| when someone has a humeral immunity decreases what 7 things are they more susceptible to |
|
Definition
pyogenic bacteria: otitis, pneumonia, meningitis, osteomyelitis
enteric: bacteria and viruses
parasites |
|
|
Term
| cell mediated i mmunity: function, general moa |
|
Definition
provide defense against intracellular microbes (phagocytosed)
T cells activate macrophages |
|
|
Term
| how is cell mediated immunity defective, what does this due to the immune system |
|
Definition
defects lead to infection by virus or intracellular microbes
can affect humeral and cell mediated immunity |
|
|
Term
| what are 3 signs of cell mediated imunity deficiency |
|
Definition
serum Ig is normal or reduced
delayed type hypersensitivity reactions to antigens reduced
follicles in parafollicular cortical regions (T cell zones) reduced |
|
|
Term
| when someone has a cell mediated immune deficiency what are 4 things they are susceptible to |
|
Definition
pneumocystisis carinii
viruses
fungi
atypical mycobacteria |
|
|
Term
| in general defect in humoral immunity leads to susceptability to ____ |
|
Definition
|
|
Term
| in general defect in cell mediated immunity leads to infection by _____ or _______ |
|
Definition
| viruses or intracellular microbes |
|
|
Term
| combined immunodeficiency diseases: onset, prognosis |
|
Definition
onset is early
prognosis is death without intervention |
|
|
Term
| unodefiencicies developed from genetic defects are classified as ________ |
|
Definition
|
|
Term
| what are three major conditions that immune deficiency can lead to |
|
Definition
susceptability to cancer
autoimmunity
susceptability to live vaccines |
|
|
Term
| what are three general process that cause immunodeficiency |
|
Definition
| lymphocyte maturation, development, activation, or effector issue |
|
|
Term
| primary immune deficiency diseases : aka, causes (3) |
|
Definition
genetic or developmental defect: at birth or inherited
myeloid or phagocyte dysfinctions
lymphoid or T/B cell dysfunctions |
|
|
Term
| what are the three types of primary immune deficiencies |
|
Definition
missing enzyme
missing cell type
non-functioning cell components |
|
|
Term
| what are 8 signs of primary immune deficiency |
|
Definition
8+ ear infections in a year
2+ sinus infections in a year
2+ months on antibiotic with little effect
2+ pneumonias in a year
failure to thrive
recurrent deep skin or organ abscess
persistant thrush after 1 yo
two or more deep seated infections
family history |
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|
Term
| secondary immune deficiency causes 6 |
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Definition
underlying disease
exposure to various agents
lymphoid malignancy
infection (HIV)
malnutrition
immune supression drugs |
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|
Term
| what are the 5 steps in B cell maturation |
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Definition
| stem cell > pro B > pre B > immature B cell > mature B cell |
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|
Term
| what are the 5 steps in T cell maturation |
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Definition
| stem cell > pro T > pre T > double positive immature T > single positive mature T cell |
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Term
| what diseases incude a defect between stem cell and pro B cell (2), where else can a defect be that produces the same set of diseases |
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Definition
stem cell and pro t cell
ADA, PNP deficiency (autosomal SCID) |
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|
Term
| what immune deficiencyes have to do with a defect between pro B and pre B cell (1), where else could the be a defect that could cause the same condition |
|
Definition
pro T and pre T cell
RAG deficiency (autosomal SCID) |
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Term
| what diseases as associated with a defect going from stem cell to pro T cell (4) |
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Definition
| ana, PNP deficiency (autosomal SCID), yc deficiency (x-linked SCID), lack of thymys (DiGeorge syndrome) |
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|
Term
| what diseases are associated with an issue with going from pre B and immature b cell |
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Definition
| Btk deficiency (x-linked agammaglobulinemia) |
|
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Term
|
Definition
| defective T cell function in combination with B or NK cell defect |
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|
Term
|
Definition
low circulating lymphocytes
thymus dosent develop
severe recurrant infections (fungi, viruses that T cells normally kill) |
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|
Term
| what are signs of SCID in an infant (4) |
|
Definition
| chronic diarrhea, pneumonia, skin and mouth lesions, opportunistic infections |
|
|
Term
| what are the three types of SCID |
|
Definition
| x-linked, adenosine deaminase deficiency (autosomal), RAG deficiency (autosomal) |
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|
Term
| x-linked SCID: disease process |
|
Definition
prevents cytokine receptor signaling during T cell development (especially IL-7)
decreased T cells, normal levels of B cells but with decreased Ig production due to lack to T cell induction |
|
|
Term
| adenosine deaminase deficiency disease process |
|
Definition
| ADA plays a role in DNA synthesis so during synthesis toxic metabolites form and lymphocytes are killed reducing B, T, and NK cells |
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|
Term
|
Definition
| congenital thymic aplasmia, in severe forms no thymus |
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|
Term
|
Definition
facial abnormalitis, hypothyroidism, congenital heart disease
normal B cells that don't make Ig due to lack of T cell induction |
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|
Term
| burton's ammaglobulinemia: aka, cause |
|
Definition
x-linked hypoammaglobulinemia
no peripherial B cells due to a defect in maturation
low or no Ig |
|
|
Term
| burton's ammaglobulinemia signs: 2 |
|
Definition
bacterial infections begining at 6-9 mo as mom's immune system weigns
due to S. pneumonia and H. influenza |
|
|
Term
| x-linked hyper IgM syndrome: lab signs |
|
Definition
IgA, IgG, IgE deficiency
lots of IgM |
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|
Term
|
Definition
defect in CD40L gene
b cells dont signal T dependatn antigens
loss of isotype switching from IgM to other Ig
T independent antigen response unaffected
lymph node has NO germinal centers |
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|
Term
| common variable immune deficiency (CIV): cause, onset age, aka and why |
|
Definition
unknown cause
symptoms appear in 20s and 30s
most common cause of hypoammaglobulenemia |
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|
Term
| CIV disease process and clinical sign |
|
Definition
decrease in antibody producing plasma cells due to lack of maturation and low Ig
B cell fail to mature into plasma cells
common for patients to develop autoimmune disorders
recurrant bacterial infections |
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|
Term
| Job syndrome: aka, cause, lab levels |
|
Definition
hyper IgE syndrome
defect in HIES gene on chromosome 4
elevated IgE, eosinophilia |
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|
Term
| Job syndrome clinical signs (5) |
|
Definition
recurrant infection
skin abscess
pneumonia
eczema
facial abnormalities
bone fragility |
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|
Term
| selected IgA deficiency: claim to fame, cause |
|
Definition
most common B cell disorder
lack of IgA on mucosal surfaces due to inability of IgA B cells to differentiate into plasma cells |
|
|
Term
| selected IgA deficiency: clinical signs (5) |
|
Definition
asymptomatic
recurrent respiratory infection
recurrent GU infection
intestinal malaborption
autoimmunity |
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|
Term
| leukocyte adhesion deficiency 1: cause, disease process, 2 clinical signs |
|
Definition
mutation in gene for B chain CD18 on B2 integrin
abseent B2 integrins causing decreased adhesion
bacterial and fungal infections |
|
|
Term
| leukocyte adhesion deficiency 2: cause |
|
Definition
| mutation in CDP fructose transporter gene which makes sialyl lewis X component of E and P selectins |
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|
Term
| leukocyte adhesion deficiency 2 disease process, 2 clinical signs |
|
Definition
deficient expression of leukocyte ligands to E and P selectins, failure of leukocyte migration
recurrant bacterial and fungal infections |
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|
Term
| chronic granulomatosus disease: cause |
|
Definition
| mutation in gene encoding components on phagocyte oxidase enzyme (most often chtochrome B558) |
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|
Term
| chronic granulomatosus disease process |
|
Definition
| defective production of ROS by NADPH oxidase in phagocytes (usually issue with superoxide microbicidal) |
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|
Term
| chronic granulomatosus disease signs (6) |
|
Definition
excessive inflammation reactions
gingivitis
swollen nodes
nonmilignant granulomas
susceptibe to bacterial infection
susceptibe to fungal infections |
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|
Term
| chediak higashi syndrome: cause |
|
Definition
| mutation in unknown gene increasing fusion of cytoplasmic granules |
|
|
Term
| chediak higashi disease process |
|
Definition
defective lysosome function in neutrophils, macrophages, dendeitic cells
defective granule function in NK cells |
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|
Term
| chrdiak higashi clinical sign |
|
Definition
| recurrent pyogenic bacterial infections |
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|
Term
| what type of hypersensitivity forms granulomas |
|
Definition
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