Immuno 3 Flashcards

1
Q

X-linked agammaglobulinemia

A

defect in B cell maturation: premature B cells can’t mature to B cells –> males don’t have circ B cells –> no igA/D/M/E and little igG –> pyogenic infxn –> tx w/ IV ab

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2
Q

selective igA immunodefic

A

no igA but other ab nml –> mucosal infxn (sinus, lungs, intestines); MOST COMMON PRIMARY IMMUNODEF; most common clinical condition = anaphylactic transfusion rxn b/c have anti-igA ab

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3
Q

CVID

A

group of d/o; dec in igG/A/sometimes M –> plasma cells can’t mature –> recurrent bacterial infxn b/c ab can’t make immune responses; affects both sexes equally

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4
Q

x-linked vs auto rec SCID

A

mutation in common gamma chain of IL7 receptor –> pro-B/T cells can’t differentiate –> no B/T cells –> no IL15 –> no NK or CD8 –> no humoral/CMI vs any mutation not on X chrm

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5
Q

ADA

A

buildup of A & G –> toxic to lymphocyte stem cells –> stem cells die before they can differentiate into B/T cells –> lymphopenia, opportunistic infxns, inc autoimmunity (LOA)

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6
Q

Omenn Syndrome

A

Hypomorphic mutation in RAG1/2 –> T cells present but not fxnal, no B cells –> abnl T cells bind to thymus self ag –> GVHD or autoimmunity

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7
Q

MHC II defic

A

B/T cells present but not fxnal; MHC II not expressed on macs or B cells –> can’t present to CD4 –> can’t activate B/T cells (MHC I can still present to CD8 but need CD4 to expand immune response) –> GI infxns

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8
Q

hyper igM vs igE

A

x-linked; mutation in CD40L on X chrm –> T cells can’t express fxnal CD40L –> can’t costim to help macs or B cells –> B cell can’t isotype switch –> stays as igM –> defects in humoral and phag responses –> susceptible to P. jirovecii vs mutation in STAT3 –> no IL17 prod –> inc igE and eos –> FATE

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9
Q

Wiskott-Aldrich syndrome

A

B/T cells present but not fxnal; Defect in WAS protein –> ineffective B cell response to T-independent polysaccharide Ags –> susceptibility to encapsulated bacteria; Defect in WAS protein –> defective formation of immune synapse –> ineffective activation of T cell by APC; classic triad = thrombocytopenia, immunodefic, eczema

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10
Q

Bare Lymphocyte Syndrome II

A

Mutations in TRANSCPXN FACTORS that regulate MHC Class II gene expression –> little/no MHC-II expressed BUT MHC-I is expressed at normal or slightly reduced levels –> can’t present in thymus –> can’t activate T cells –> can’t activate B cells –> recurrent GI infxns, no DTH

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11
Q

TREC

A

excised DNA that help make TCRs; high TREC –> many TCR, low TREC –> few TCR; used to screen newborns for thymic activity

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12
Q

DiGeorge

A

microdeletion of 22q11.2 –> thymic aplasia –> no T cells –> viral, fungal, mycobacterial infxn; CATCH22

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13
Q

IL12 defic

A

Mutation in p35/40 –> fxnal IL12 heterodimer
Mutation in IL12 receptor –> IL12 can’t bind –> dec CD4 Th1 and CD8 –> dec IFNγ –> tx w/ exogenous IFNγ

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14
Q

MHC I defic

A

mutation in TAP –> can’t load peptide onto MHC I/bare surface –> no CD8 –> recurrent viral infxn; inc NK and gamma/delta T cells to compensate

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15
Q

malnutrition causing immunodef

A

lower CD4 –> low CD4:CD8 ratio –> impaired inflamm and wound healing, reduced phag

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16
Q

age causing immunodef

A

immunosenescence –> inc neoplasia to ca, susceptibility to dz, dec inflamm response b/c low IL1/6; dec stem cell prolif, thymic involution, dec innate and adaptive immunity

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17
Q

Iatrogenic (Drug-Induced) Immunodeficiency: targeted therapies

A

corticosteroids and immunotherapy (inhibits effector fxn), cytotoxic drugs (blocks B/T cell prolif and diff), chemotherapy (dmgs hematopoietic cells by altering DNA structure –> anemia, lymphopenia, neutropenia)

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18
Q

Iatrogenic (Drug-Induced) Immunodeficiency: non-specific therapies

A

Cyclosporine, gold, penicillamine, anti-epileptics –> induce transient igA defic b/c adverse drug rxn

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19
Q

alc causing immunodef

A

alc –> reduced intestinal absorption of proteins and micronutrient –> altered microbiome
releases endotoxin in GI –> Kupffer cells release ROS and cytokines –> destroy hepatocytes
stimulates hypothalamic-pituitary-adrenal axis –> release immunosuppressive corticosteroids –> reduced phagocyte chemotaxis and phagocytic/killing; diff in both sexes: males release testosterone –> immunosuppressive, females have dec estrogen –> immunosuppressive, and they have dec metab for alc –> liver cirrhosis

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20
Q

Infection-Induced Immunodeficiency

A

produces a transient suppression except retroviruses

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21
Q

another name for artificial passive immunity. who’s at risk?

A

passive immunization, immunoprophylaxis. ppl not exposed to ag before, at risk for severe/life threatening dz, immuno incompetent

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22
Q

salk vs sabin

A

IPV –> igGAM but not mucosal igA vs OPV –> igGAM and mucosal igA

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23
Q

bacterial vs viral vs parasitic vaccines

A

toxins/attachment/motility factors –> T dep B cell ag; polysaccharide capsules for T indep B cell ag; goal = conjugate capsule to T dep B cell ag vs need to induce CMI vs none yet b/c too many life stages but we have cheap antiparasitic drugs

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24
Q

vaccine routes: parenteral vs mucosal

A

IM, intradermal –> igG and CMI, little igA vs intranasal, a little more igA

25
passive vs active immunity
short lived, preformed ab, immediate protection, good for immunodef ppl, bad w/ rpted doses, no mem vs long lasting, need time to make response, lag, bad for immunodef ppl, rpt doses = good, mem
26
early vs late phase of T1HS
igE binds to allergen --> P ITAM --> mast cell degran/phag --> histamine, PG/LT, enzymes vs cytokines recruit Th2, mac, eos --> release toxic proteins/enzymes, ROS, pro-inflamm cytokines, eiconasoids --> chronic inflamm --> fibrosis
27
which type of DCs found in food allergies?
immunogenic DC > tolerogenic DC/nml
28
what does SCIT do?
dec igE, inc igG & Treg
29
key sxs and dx for acute hemolytic anemia
anemia, BLISTERING OF SKIN/MUCOUS MEMBRANES, jaundice. dx by COOMBS TEST or immunocytochem
30
key sx for pernicious anemia
tingly/numb hands/feet
31
key sx for pemphigus vulgaris vs goodpasture's
BLISTERING OF SKIN/MUCOUS MEMBRANES vs dysuria/hematuria, hemoptysis/cough/dyspnea
32
key sx for graves dz
goiter, tachy, exophthalmos, itchy, sweat, tremors
33
acute vs chronic serum sickness
1 large ag dose --> ab prod --> phag vs chronic exposure to ag --> antigenemia
34
sensitization of T1HS vs T4HS
allergen to PRR --> Th2 cells --> IL4/5/13 --> isotype switch to igE --> igE bind to mast vs allergen to PRR --> Th1/2/17 --> memory cells
35
TRALI
donor plasma ab react to recip ag --> C', cytokines --> fever/chills, hypoTN, frothy sputum, HYPOXIA --> tx by give O2 or mechanical ventilation
36
erythroblastosis fetalis vs hydrops fetalis
mom's anti-Rh ab attack baby's Rh on RBC --> RBC lysis --> high bilirubin --> anemia and jaundice in fetus vs DAMPs bind to PRR --> cytokines --> edema in fetus
37
most common GVHD?
bone marrow transplant
38
diff b/w serology prescreen vs serum based assay for tissue type vs cross match screen
is donor infected w/ anything? vs will recip serum ab attack donor MHC? vs will recip serum ab attack donor cells?
39
hyperacute vs acute vs chronic stages of allorecognition
min to hrs; more for xenotransplants; ab --> C' --> clot --> inflam, endothelial dmg --> tx by removal vs days to wks, highest risk = 3mo; ab, CD4 (IL2; IFNa/B for DC, MHC I/II; IFNy for mac/NK, MHC II; TNFa/B for MHC I), CD8 (cytolysis) --> tx by immunosuppressant vs mo to yrs; slow progressive tissue destruction by inflamm --> ab, mac, CD4/8 --> fibroblasts --> fibrosis; highest susceptibility in sm arteries and airways
40
how to est maternal fetal tolerance?
amniotic fluid: AFP placenta: HLAG, IL10/TGFB/IDO, hCG
41
ex of immunosuppressants
corticosteroids, cyclosporine, ab/biologics (block cytok binding), mTOR inhibitor/cytotoxic drugs (block prolif)
42
Arthus rxn vs serum sickness
IC in vessel/tissue --> inflamm, PLT, ischemia, necrosis vs IC in kid, vessel, joint --> glomerulonephritis, vasculitis, arthritis
43
central tolerance vs peripheral tolerance vs suppression
delete alloreactive T cells vs induce anergy of alloreactive T cells vs inc Treg
44
transfusion rxn vs allergic transfusion rxn
T2HS d/t mismatched blood type by human error --> transfused foreign RBC = destroyed by C’ mediated lysis/phag/ADCC --> fever/chills, tachy, change in bp, intravascular clots, lower back pain b/c blood entering kidneys --> stop transfusion and replace donor blood w/ nml saline vs recip = presensitized to donor RBC --> igE = activated upon next exposure --> stop transfusion
45
ARF
ab bind to S. pyogenes M protein --> circ --> bind to myosin in heart, joints, vessels --> C’ --> tissue dmg --> heart palpitations, swollen red joints/uncontrolled movements, stomach pain
46
key pathogenesis of autoimmune thrombocytopenic purpura?
bind to PLT --> activate splenic/hepatic mac --> clearance of spleen and liver --> low PLT
47
contact dermatitis
ag to DC --> Th1 --> mem --> activated mem --> IFNy --> tissue dmg
48
rheumatoid arthritis
broken tolerance --> synovial ag to Th1 --> IFNy and TNF --> mast, osteoclast, chondrocytes --> dmg synovium, bone, cartilage
49
TIDM
broken tolerance --> CD4/8 attack pancreatic B cells --> IL2/IFNy, cytolysis/perforin/granzyme/apop
50
DC based vs T cell based of cell based immunotherapy
active; Apheresis and remove myeloid cells --> make “right” DC --> make “right” Th1 and CD8 cells in vivo --> make “right” cytokines in vivo --> inhibit cytokines that favor tolerogenic DCs --> admin to pt vs passive; Remove T cells --> remove “natural” TCR --> replace w/ tumor-specific recombinant TCR --> make “right” cytokines in vivo; Chimeric TCR = Fab of tumor specific ab + CD3 tail
51
what does receptor blockade do?
prevents immune system from turning off
52
advantages of immunotherapy?
specifically targets --> no lost anti-ca activity --> mem
53
complication of immunotherapy tx: latent infxn
when immune mediators of infectious agent = removed --> reinfxn; ex: TNF targeted immunotherapies remove TNF --> dissoc of TB granuloma --> TB wreaks havoc
54
immune checkpoints and their inhibitors
CTLA-4 bind to CD80/86, or PD1 binds to PDL1 --> turns off T cell; inhibitors (antiCTLA4/PD1/PDL1) keep T cell on. ICI can break tolerance --> autoimmune dz --> re/appearance of autoreactive T cells
55
BiTE
Bispecific T cell Engager: take Fab of tumor specific ab and antiCD3, link em together --> forces T cell and tumor cell binding; ex: CD3 T cell w/ CD19 tumor cell (blincyto)
56
stages of tumor progression
dysplasia (growth factors, apop inhibition), invasion, angiogenesis, metastasis (altered expression of cell adhesion molec, chemokines/receptors)
57
3 stages of immunity fighting tumors
1) elim: by CD8, NK, mac/DC, IL12, IFNy 2) equil: by CD8, ab, CD4 Th1, IL12, IFNy; immunoedit and ca edit 3) escape: by tolerogenic DC, monocyte-derived suppressor cells, Treg; immunoevasion (B/T exhaustion) and immunosuppression (turn off immune response w/ FasL, CTLA4, PD1)
58
graft and tumor rejection = mostly done by which cells?
CD8
59
blood A/B ag specificity depends on?
single terminal sugar residue