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 |
|
|