Term
| what are the causes of secondary immunodeficiency (10) |
|
Definition
T cell activity defect
immunosupressive therapy
spleen surgery/disease
nutrition/malnutrition
trauma
age
microbe infection
malignancy
biocehmical homeostasis imbalance
protein loss |
|
|
Term
| what are the types of immunosupressive therapy (5) |
|
Definition
ctotoxic chemotherapy for malignancy
treatment of autoimmune disease
marrow albation prior to transplant
treatment of prophylaxis for solid organ transplant
immunosupressive drugs |
|
|
Term
| what are the immunosupressive drugs (4) |
|
Definition
cyclosporine A and tacrolimus
corticosteroids
mnolclonial antibody to cytokine (TNF) |
|
|
Term
| what do cyclosporine A and tacrolimus do |
|
Definition
block calcineurin protein which activates T cells
disrupts signal transduction |
|
|
Term
| what do corticosteroids do (3) |
|
Definition
| inhibit cytokine production, affect antigen presentation, reduce peripherial lymph nodes |
|
|
Term
| what does monoclonal antibodies to a cytokine do to the body |
|
Definition
| interleukin or receptor dysruption |
|
|
Term
| what does a splenectomy do to the immune system |
|
Definition
| reduced response to t independent antigens and to encapsulated bacteria |
|
|
Term
| what are the major areas of nutrition deficit that affect the immune system (9) |
|
Definition
| proteins, glutamine, iron, zinc, selenium, vitamin A, B6, C, E |
|
|
Term
| overall, what does changes does malnutrition cause in the immune system |
|
Definition
| decreased circulating T cells, increased NK cells, increased Ig levels but decreased response, less cells in lymph organs, increased incidence of infection |
|
|
Term
| what are the types of trauma that can dysrupt the immune system (3 + 3 subcategories) |
|
Definition
| mechanical, burn, enviromental (radiation: UV, chemotherapy, ionizing) |
|
|
Term
| what does mechanical trauma do to the immune system |
|
Definition
| increase inflammatory cytokines (IL-1, TNF) |
|
|
Term
| what do burns to do the immune system |
|
Definition
| barrier dysruption and serum protein leaking increases infection risk |
|
|
Term
| what are the types of radiation, what do they do the immune system (3) |
|
Definition
UV: skin cancer, decrease lymphocytes, mast, and dendritic cells
chemotherapy: cytotoxic to rapidly dividing cells (like immune stem cells in marrow)
ionizing (x-ray, y-ray): impairs cell division (malignancy, mutation) |
|
|
Term
| what does HIV do to the immune system |
|
Definition
| infects CD4 and is cytotoxic |
|
|
Term
| what does measles do to the immune system (3), what usually causes death of these patients |
|
Definition
T cell lymphopenia (deletion of T cell areas of spleen, nodes, decreased antibodies to to T cell infection)
CMI supression
interfere with signaling
death due to co-infections |
|
|
Term
| what does CMV do to the immune system |
|
Definition
| transporter associated with antigen processing (TAP) transfers antigen to the ER. this is prevented so MHC-1 never gets to surface |
|
|
Term
| what does herpes change in the immune system (2) |
|
Definition
MHC-1 cannot get to the ER and is not displayed
reduced INFy production |
|
|
Term
| what does EBV do to the immune system |
|
Definition
| IL-10 analog inhibits Th1 development |
|
|
Term
| what bacteria (or categories of bacteria) cause immunosupression. what is their weapon (4) |
|
Definition
S. aureus: superantigen PVL
gram negative: endotoxin causes spesis
gram positive cocci: toxin causes toxic shock
mycobacterium |
|
|
Term
| how does mycobacterium cause immunosupression (3) |
|
Definition
interfere with phagolysome fusion to lysosome preventing phagocytosis of itself and other microbes
replicates in phagocytes
increased risk for secondary infection |
|
|
Term
| what are two categories of malignancy that cause immunosupression |
|
Definition
| leukemia and reduced Ig production |
|
|
Term
|
Definition
proliferation of abnormal cells leading to functional issues and crowding
cancer of immune system |
|
|
Term
| what are examples of reduged Ig production malignancies that cause immunosupression, give a brief description of them (4) |
|
Definition
hodgkins disease: lymphoid tumor
chronic lymphocytic leukemia: B cell tumor
multiple myeloma: many monoclonial antibodies. marrow tumor
waldenstorm macroglobulinemia: lymphoplascytic lymphoma, increased IgM due to tumor in IgM secreting B cell |
|
|
Term
| what are two conditions that can cause biochemical homeostasis imbalance |
|
Definition
| diabetes mellitus and hepatic insufficiency / cirrhosis |
|
|
Term
| how does diabetes mellitus cause immunosupression (2) |
|
Definition
reduced neutrophil killing ability due to fingal infection and hyperglycemia
bad peripherial circulation causes skin ulceration and reduced neutrophil delivery |
|
|
Term
| how does hepatic insufficiency / cirrhosis cause immunosupression (3) |
|
Definition
serum protein issues
increased glycocorticoids cause immune dysfunction
blood shunting decreases kupffer cell exposure to blood |
|
|
Term
| what are disorers that cause protein loss |
|
Definition
nephrotic syndrome (can come from immunosupressive drugs)
severe dermititis (proteins leak out of skin)
peritoneal dialysis across membrane (treatment for chronic renal disease)
enteropathy (leaking of proteins into GI lumen) |
|
|
Term
| what are the signs of a primary immunodeficiency (17) |
|
Definition
mostly in kids
after exclusion of organ, allergy, asthma, anatomic abnormality
normal growth and development
healthy between episodes
quick response to treatment
recurent infection or abcess (especially GI, respiratory)
antibody therapy isnt working
unusual opportunistic infections
family history, especialy in males
structural abnormality
failure of infant to thribe
hepatosplenomeagly
malignancies
chronic thrush, rash, riarrhea
reduced lymphoid tissue |
|
|
Term
| what are the B cell primary deficiencies (4) |
|
Definition
ataxia telangiectasia
burton's ammaglobulinemia
IgA defieicney
common variable immunodeficiency |
|
|
Term
| what are complement, cytokine, co-stimulatory, and signal transduction deficiencies (9) |
|
Definition
hereditary angioneurotic edema
C2 deficiency
C3 activation defect
factor H or I defect
C3-C4 defect
C5-C9 defect
MAC attach interruption
INFy receptor defect
decay accelerating factor deficiency (CD59) |
|
|
Term
| what are phagocyte primary deficiencies (6) |
|
Definition
CD18 mutation
leukocyte adhesion deficiency
congenital agranullocytosis
chronic granulomatous disease
chediak higashi syndrome
respiratory burst |
|
|
Term
| what are T cell primary deficiencies (15) |
|
Definition
SCID
CD40/CD154 x-linked hyper IgM
wiskott aldrich syndrome
adenosine deaminase / purine nucleotide phosphorlyase defect
bare lymphocyte syndrome
thymus maturation disorders
stem cell disorders
surface receptor disorders
signal transduction disorders
diGeorge syndrome
RAG gene mutation
omen syndrome
IL-2 production or receptor issues
CD3 zeta defect
ZAP 70 phosphotyrosine kinase defect |
|
|
Term
| ataxia telangiectasia: inheritence, issue, symptoms |
|
Definition
autosomal
cant repair DNA breaks in T and b cells, purkinje fiber movement issues in development and vision
loss of balance, widened capillaries, increased malignancy, retarted growth, immunodefects of superimmunoglobins |
|
|
Term
| what is the cause of x-linked (burton's) agammaglobulinemia (3) |
|
Definition
defect in burtons protein tyrosine kinase (BTK) CD40
intracellular signal for pre B to pro B cell stopped, Ig reduced |
|
|
Term
| what are the effects of x-linked (burton's) agammaglobulinemia (2) |
|
Definition
| increased respiratory, meningitis, and GI infection, increased vulnerability to bacteria with polysaccharide capsules |
|
|
Term
| treatment of x-linked (burton's) agammaglobulinemia (2) |
|
Definition
| injection of gamma globulin or antibody from plasma of healthy donors |
|
|
Term
| what is the cause of an IgA deficiency |
|
Definition
IgA precursor cell fails to mature due to 6 chromosome 14 deletion options
IgM and IgG normal |
|
|
Term
| what are the effects / symptoms of an IgA deficiency (2) |
|
Definition
increased GI, respiratory infection, allergy, CNS disorders, autoimmune diseases
may produce anti-IgA antibody causes anaphylaxis |
|
|
Term
| what is the signs of common variable immunodeficiency (CVID) (3) |
|
Definition
presents a little after birth because mom's IgG help
recurrent respiratory infection, low IgG |
|
|
Term
| what are general signs of a B cell primary deficiency (3) |
|
Definition
resist many pathogens and treated by antibiotics for rest
bronchiectasis (loss of elasticity due to chronic inflammation), chronic lung disease
tissue damage due to treatment causing protease release from phagocytes and abcteria |
|
|
Term
| how are B cell primary deficiencies detected (4) |
|
Definition
reduced Ig titer following vaccine
Ig quantitation
reduced B cell numbers
flow cytometry cell count for monclonial antibody to CD19 |
|
|
Term
| what are the general signs of a complement,cytokine, co-stimulatory, or signal transduction primary deficiency (4) |
|
Definition
| increased alternate and mannose-lectin binding pathway, bacterial infection, autoimmune diseases due to inability to destory immune complexes, accumulation of immune complexes |
|
|
Term
| herditary angioneurotic edema: causse and effect |
|
Definition
C1 inhibitor (C1INH) leaks and activates classic complement pathway
overproduction of vasoactive C2a causes edema and epiglottal swelling |
|
|
Term
| C3 activation defect: cause, effect |
|
Definition
missing protein
decrease in phagocytosis, susceptibility to pyrogenic infections |
|
|
Term
| factor H or I defect effects |
|
Definition
conversion of C3 to C3b is unchecked and C3 stock pile is depleted due to alternate pathway activation
decreased MAC attack |
|
|
Term
| what are the isgns of a C3-C4 defect |
|
Definition
cant clear immune complexes because C3/4 attach to CR1 on RBC to capture complexes
inflammation, tissue damage |
|
|
Term
| what does C5-9 defect increase susceptability to |
|
Definition
|
|
Term
| how is an interruption of the MAC attack immunodeficiency treated (2) |
|
Definition
| gamma globulin injections, GM-CSF injection to stimulate phagocyte differentation |
|
|
Term
| what is the cause and effects of decay accelerating factor (DAF CD59) deficiency |
|
Definition
not protected from activating alternate pathway of complement activation
complement mediated lysis of RBC causes paroxymal nocturnal hemoglobinuria |
|
|
Term
| how are complement and cytokine disorders detected (5) |
|
Definition
complement or regulator levels
analysis of components
opsonic index
cytokine levels
Ig levels |
|
|
Term
| mutation of BD18: inheritance, what it does to body, what disease it causes |
|
Definition
autosomal recessive
codes for B subunit of leukocyte integrins (CR2, CR4, LFA-1) adhesion molecules
causes leukocyte adhesion deficiency |
|
|
Term
| what are the effects of leukocyte adhesion deficiency on the body (6) |
|
Definition
phagocytes cannot leave blood and enter infection site
persistant infection, poor wound healing, gum inflammation, poor response to antibiotics
leathal without marrow transplant |
|
|
Term
| what is the cause of congrnital agranulocytosis |
|
Definition
| decreased GM-CSF causing decreased neutriphils and macrophages |
|
|
Term
| what is the cause of chronic granulomatous disease |
|
Definition
| phagocytes cant make superodixde radical due to mutation in NADPH oxidase enzyme (myeloperoxidase and G6PDH) |
|
|
Term
| what are the effects of chronic granulomatous disease (3) |
|
Definition
| chronic bacterial infection, granuloma, chediak-higashi syndrome |
|
|
Term
| what is the tretment for chronic granulomatous disease |
|
Definition
| antimicrovirals, gamma globulin, marrow transplant, gene transfer |
|
|
Term
| what is the cause of chediak-higashi syndrome (3) |
|
Definition
defective CHS1 gene on 1q42043 causes deficiency or G6PDH or myeloperoxidase
phagocytosed material not delivered to the lysosome (poor granular trafficing) due to vesicle fusion defect (cytiskeleton proteis missing) |
|
|
Term
| what are the symptoms of chediak-higashi syndrome (6) |
|
Definition
| albnism, peripherial neuropathy, lysosomal granules, reduced chemotaxis and phagocytosis due to decreased neutrophils, increased pyogenic infection, decreased platelets causing bleeding disorders |
|
|
Term
| what causes respiratory burst (2) |
|
Definition
high NO due to a NADPH oxidase enzyme deficiency reduces vascular tone and CO and contributes to the low BP of septic shock
high NO acts as messenger ot promots T cells causing chronic inflammation |
|
|
Term
| how is a phagocyte disorder detected |
|
Definition
nutroblue tetraolium reaction: yellow to purple in presence of ROS
respiratory burst assay |
|
|
Term
| how is a respiratory bust assay done |
|
Definition
WBC incubated with dihydrorhodamine and catalyase
stimulated with phorbol 12-myristase 13 acetate
dihydrprhydoamine oxidation or rhodamine by peroxidase causes green floursence
measured by flow cytometry |
|
|
Term
| what are some causes of SCID (8) |
|
Definition
no T cell dependent antibody response or cell mediated immune response. B cell as a result are not fuctional
X chromosome gamma chain causes JAK3 kinase production which codes for cytokine receptors (IL-2, 3, 7, 9, 15) decreasing signal transduction
adenosine deaminase/purine nucleotide phosphorlyase defect
bare lymphocyte syndrome (HLA-II only)
stem cell disorders
RAG gene mutation
IL-2 production or receptor issues
CD3 zeta issues |
|
|
Term
| CD154/Cd40 x-linked hyper IgM: inheritance (2), defect (2) |
|
Definition
autosomal dominant (sometimes recessive)
defects
cutidine deaminade (AID) defect.
CD40 on T cells is not activating B cells, B cells cant change to plasma cells |
|
|
Term
|
Definition
no specific antibodies made, only IgM
no hypermutations to make better antibodies
no memory cells
recurrent infection in respiratory and GI
anemia
lymphoma
autoimmunity
no germinal centers
no leukocytes increase in infection
neutropenia: blisters in mouth and throat |
|
|
Term
|
Definition
| galla globulin, antibiotics, GM-CSF |
|
|
Term
| cause of wiskott aldrich syndrome |
|
Definition
| gene on X chromosome codes for WASP protein which reogranizes cytoskeleton before T cell delivers cytokines to B cell, macrophages, or target cells. impairs plateles and lymphocytes |
|
|
Term
| symptoms / effects of wiskott aldrich syndrome |
|
Definition
| thrombocytropenia, GI bleeding, respiratory infection, ecsemia, cancer, low polysaccharide antibody response (ABO antibodies) |
|
|
Term
| adenosine deaminase (ADA) or purine nucleotide phosphorlyase (PNP) defect: inheritance, defective process, cause of toxicity |
|
Definition
autosomal recessive
enzymes in purine degration missing, causes nucleotide metabolites (dATP/dGTP) to build up and hurt developing T and B stem cells |
|
|
Term
| treatment of ADA/PNP deficiency (3) |
|
Definition
| RBC transfusion, RBC enzyme replacement, gene therapy (insertion of viral vector into patient T cells) |
|
|
Term
| what are the types of bare lymphocyte syndrome, what are common symptoms |
|
Definition
| HLA-I or HLA-II deficiency |
|
|
Term
| how does a HLA-II deficiency occur, what does this cause |
|
Definition
CD4 cannot develop due to defect in transcription regulators for HLA-II (DP, DQ, DR)
transactivator CIITA and pomotor building protein RFX are the ones messed up
causes SCID |
|
|
Term
| how does HLA-I deficiency occur, what does this cause |
|
Definition
gene to tapsin (TAP) peptide transporter is defective and delivery of peptides to ER where they would bind to make HLA-I is stopped
causes CD8 loss, NOT SCID |
|
|
Term
| what are the effects of DiGeorge (7) |
|
Definition
CMi reduced allowing infection
T dependent antigen response in tact
cardiac, facial, thymus, cleft, hypocalcemia abnormalities |
|
|
Term
| what microbes infect people with DiGeorge syndrom (8) |
|
Definition
| mycobacteria, listeria, salmonella, viruses, CMV, attenuated vaccine, canidia, pneumocystitis carinii |
|
|
Term
| what are the causes of the symptoms of DiGeorge syndrome (4) |
|
Definition
3rd and 4th pharyngeal pouches (for thymus, parotid, aorta and craniofacial) malformation
chromosome translocation
thymic aplasia
T cells dont mature enough to function |
|
|
Term
| what structures does a RAG gene mutation mess up (3) |
|
Definition
| TCR and BCR and editing of autoreactive receptors |
|
|
Term
| what is the cause of omen syndrome |
|
Definition
| less severe RAG geme mutations, partial activity |
|
|
Term
| what issues come from IL-2 production or receptor issues (3) |
|
Definition
cant activate T cell
gamma chain issues
SCID |
|
|
Term
| explain what ZAP 70 and CD3 zeta normally do |
|
Definition
CD3 transmits signals from TCR to cytoplasmic tyrosine kinase
CD3/CD4/CD8 signals combine to stimulate T cell functions
sometimes when zeta part of CD3 is used and ZAP 70 is stimulates
ZAP 70 initiates gene expression, secondary messengers, PKC, etc |
|
|
Term
| what are the effects of a CD3 zeta defect (3) |
|
Definition
T cell development defective
SCID
signal transduction issues |
|
|
Term
| what are the effects of ZAP 70 phosphotyrosine kinase defect (2) |
|
Definition
faulty interaction with CD3 zeta
poor CD8 development |
|
|
Term
| how are T cell disorders detected (4) |
|
Definition
flow cytometry cell count for antibody to CD3, 4, 5, 16, 56
reduced CD3, 4, 8
reduced DTH to tuberculin, canidia, trichophytin, mumps
mitogen responses |
|
|
Term
| what are mitogen responses |
|
Definition
| acrivation and proliferation of lymphocytes to phytohemagglutinin, pokeweek, concanavalin A |
|
|
Term
| metagenomics: define, use |
|
Definition
DNA sequencing of member organisms from samples in a non-biased manner, not targeting a specific species
not suitable for clinical setting, low sensitivity, time constraints, cost
might save lives in an outbreak where standard culture based tests dont exist |
|
|
Term
| what are the two ways to perform agglutination |
|
Definition
| hemagglutination, latex particle |
|
|
Term
| hemagglutination: process, example |
|
Definition
antigen is on RBC and antibodies to surface antigen join RBC.
antigens include ABO, Rh, blood groups
example: inhibition for antibody to influenza |
|
|
Term
| explain how to test with a latex particle, give examples |
|
Definition
latex particle is coated with antibodies and specimine is mixed in, cross linking of antibody causes visible agglutination
examples: Rh to IgM/IgG, HCG in urine, rotavirus in stool, influenza in throat swab |
|
|
Term
| how do you do an ELIZA test, what is a common example |
|
Definition
need to know antibody
bind, wash, label, and read
rapid strep test |
|
|
Term
| explain how a rapid strep test works |
|
Definition
S. pyogens cell wall antigen extracted from throat swab is added to antibody strip via capillary action.
bound antigen reacts with second enzyme labeled antibody to make a visible color |
|
|
Term
| explain how flow cytometry works |
|
Definition
antibody tp surface molecule is tagged with flouresence
cell passes through laser light
excited dye is detected electronically |
|
|
Term
| how is a polyclonial antibody used for testing |
|
Definition
antibody injected into suitable animal
variety of antibodies to different epitopes of antigen |
|
|
Term
| how is a monoclonial antibody used for testing |
|
Definition
antibody to a single determinant
highly specific |
|
|
Term
| what does a stain tell us about a microbe |
|
Definition
| preliminary information: bacterial, fungi, parasite |
|
|
Term
| what does a culture tell us about a microbe |
|
Definition
| artificial, tissue, animal, bacterial, fulgal, viral |
|
|
Term
| what does a tissue culture tell us about a microbe, example |
|
Definition
microbes change the tissue their in
viruses cause multinucleate cells in epithelium |
|
|
Term
| what does a serological culture tell us about a mcirobe |
|
Definition
| detects antigen or antibody |
|
|
Term
| from what categories can a microbe be identified using simple characteristics and biological properities |
|
Definition
simple characteristics
biochemical properities
FA analysis of the cell wall
determining antibotic susceptibility
Mass Spec |
|
|
Term
| what are simple characteristics of microbes |
|
Definition
gram reactions
morphology: rod, coccus, pairs, chains
anaerobic, aerobic
growth requirements (simple/fastidious) |
|
|
Term
| what are biochemical properities of microbes |
|
Definition
enzymes made
oxidation or fermentation of sugars
substrates used for growth |
|
|
Term
| explain how a mass spec works |
|
Definition
patterns of protein detected directly from bacteria
applied to metal plate, irridated with laser, matrix absorbs laser, vaporizes sample (ionization) giving it a charge, electric fields guide ions to flight mass spectrometer, ions separated according to mass charge ratio
quantity of each ion measured |
|
|
Term
| what are the disadvantages of antibody detection methods for diagnosis |
|
Definition
must know antigen
retrospective: it takes 2-4 weeks for IgG to develop then sustain in infection
positive test only indicates contact with microbe at some point in time
IgM qithin 7-10 days of infection can be seen as active but later are inactive |
|
|
Term
|
Definition
|
|
Term
| what can western blod test for |
|
Definition
| confirm tests for lyme disease and HIV |
|
|
Term
| what can anti-streptolysin O test for |
|
Definition
|
|
Term
| how do you assess the host defense system |
|
Definition
detect opsonic activity of complement
respiratory burst
nitroblue tetrazolium (NBT) test
lymphocyte testing via polyclonial stimulators
lymphocyte testing via surface molecules
ELiSPOT technique
using ability of Tc and NK to attack a target |
|
|
Term
| how do you detect opsonic activity of complement |
|
Definition
| measure serum sample ability to facilitate uptake microbal particle by phagocytes |
|
|
Term
| how does a nitroblue tetrazolium test work |
|
Definition
assess ability of neutrophils to become phagocytitic and reduce molecular O2
NBT forms complex with heparin or fibrogen and neutrophils phagocytose them
dye is taken into neutrophils in substitute of O2 |
|
|
Term
| how is a lymphocyte tested via surface molecules |
|
Definition
| immunoflourescent techniques |
|
|
Term
|
Definition
identify lymphocyte secreting antibodies (B cells) or cytokines (T cells)
secreted products are impregnated with antigen, secreted product is identified via ELIZA |
|
|
Term
| how are Tc and NK used to assess host defenses |
|
Definition
make radioactive isotope with 51Cr and put it into damaged cells
have Tc cells eat them and release isotope |
|
|
Term
| what are examples of tests that use latex particles with antibodies |
|
Definition
| rapid strep test, flow cytometry, tissue antigen, antigen in clinical specimen |
|
|
