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HDM (Sophia's) > Things to Know from Review Questions > Flashcards

Things to Know from Review Questions Flashcards

1
Q

What are the 5 clinical hallmarks of LAD?

A

delayed separation of umbilical cord
omphalitis (infection of umbilical stump)
severe recurrent infections w/ no pus formation
high WBC count
skin ulcers and gingivitis

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2
Q

What is the most frequently diagnosed primary phagocytic immunodeficiency?

A

Chronic granulomatous disease

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3
Q

What makes up the C3 convertase in the alternative complement pathway?

A

C3b+Bb

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4
Q

What would you see in a B cell-related deficit?

A

low B cells - if serum immunoglobulins are normal –> probably an innate immune problem

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5
Q

Which Ig has the shortest half life?

A

IgE

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6
Q

In what disease discussed in the CIS would there be 2x normal levels of neutrophils?

A

LAD

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7
Q

What is LAD-III caused by?

A

defective activation-dependent signaling of beta2 integrins –> similar defects as in LAD-1

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8
Q

What is NK cell killing described as?

A

extracellular

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9
Q

What is used to positively check TCRs in t cell development?

A

Tissue specific antigens on MHC I and II

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10
Q

What is activated in the lectin complement pathway?

A

MASP 1 and 2

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11
Q

In the MHC class I path of Ag presentation, how do cytokines affect proteosomes?

A

Enhances their ability to cleave viral proteins into the size and shape necessary for MHC I

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12
Q

Which Ig is in the smallest concentration in serum?

A

IgD

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13
Q

What bacteria often cause LAD I?

A

staphylococcus aureus and gram-negative bacilli

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14
Q

What is immune tolerance?

What cells does it occur in?

A

failure to mount a response to an antigen - only occurs in lymphocytes w/ Ag-specific receptors

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15
Q

What would you see in T cell-related deficit?

A

infection and even death due to live vaccinations

in addition to frequent infections, etc

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16
Q

What makes up the C3 convertase in the classical pathway?

A

C2a+C4b

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17
Q

What innate immune cell is involved in hypersensitivity reactions?
What happens with this?

A

mast cells

cross-link high affinity IgE receptor –> MC releases histamine, serotonin, etc

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18
Q

What cells undertake positive selection of T cells?

A

medullary thymic epithelial cells

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19
Q

What does the severity of LAD depend on?

A

degree of CD18 deficiency
severe = <2% normal CD18
mild to moderate = 2-30% normal CD18

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20
Q

What is the biochemical cause of CGD?

A

enzymatic deficiency of NADPH oxidase in phagocytes –> can’t make superoxide anion and O2 radicals –> can’t phagocytose

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21
Q

What is the first and only Ab made produced in a fetus?

A

IgM

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22
Q

What happens to IL-23 and IL-17 axis in LAD I?

A

neutrophils never get to infection so IL23 and IL17 never decrease –> chronic inflammation

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23
Q

Which Ig is involved in helminth parasitic defense?

A

IgE

also involved in allergies

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24
Q

What are the adaptive immunodeficiencies?

A

combined cellular and Ab = 15%
Cellular = 5%
Ab = 65%

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25
Q

What 2 membrane-bound Abs and cell marker does a mature B cell express?

A

IgM, IgD, and CD19

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26
Q

What is the idiotype of an Ab?

A

antigen-binding site = Vh + VL

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27
Q

In LAD, where do the recurrent infections generally occur?

A

oral and genital mucosa
skin
GI
respiratory tracts

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28
Q

What is a granuloma?

A

build-up of unregulated inflammation - often see at wound sites in CGD

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29
Q

What complements induce vasodilation and smooth muscle contraction?

A

C3a, C4a, C5a

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30
Q

Of the 3 tonsil types (palatine, pharyngeal, and lingual), which one has no definitive capsule?

A

lingual tonsils

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31
Q

What type of lymphoid nodule has a germinal center and mantle/corona?

A

secondary lymphoid nodule

primary lymphoid nodules don’t have this yet

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32
Q

What are the two major types of CGD?

A

X linked = most common form

Autosomal recessive CGD

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33
Q

What are the two Igs on naive B cells?

A

IgM and IgD

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34
Q

What is a J chain?

A

the part that connects the two parts of an IgA dimer

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35
Q

Which immunoglobulin mediates the release of mediators from mast cells and basophils when exposed to an allergen?

A

IgE

36
Q

Why does CGD cause granulomas?

A

thought to be due to failure to degrade chemoattractants and antigens –> persistent neutrophil accumulation

impaired killing of microbes –> persistent cell-mediated immune activation

37
Q

What do pts with lad NOT have increase susceptibility to?

A

viral infections

38
Q

What complements induce solubilization and clearance of immunocomplexes?

A

C3a, C3b

39
Q

What complements induce opsonization?

A

C3b, C3bi

40
Q

Which Ig is a pentamer?

A

IgM

41
Q

What is first in innate immune response, TLR activations, or NK cell activation?

A

TLR activation

42
Q

What are warning signs of an immunodeficiency disorder?

A 
8+ ear infections in one year
a bunch of other infections
deep-seated or recurrent infections
need for IV antibiotics to clear infections
family history of ID
43
Q

What test do you use to check for chronic CGD?

What does a positive test look like?

A

nitroblue tetrazolium (NBT) test

cells w/ CGD won’t stain blue; carriers will be half and half

44
Q

What do both activated NK cells and CD4+ T cells release to enhance MHC class II expression?

A

IFN gamma

45
Q

What is LAD-II caused by?

A

impaired adhesive functions of P-selectin glycoprotein ligand 1 (PSGL-1)

mutation in a fucose transporter –> defective fucosylation of PSGL-1

46
Q

What are the innate immunodeficiencies?

A

phagocytic cell = 10%

complement = 5%

47
Q

Why do pts with lad I have elevated C-reactive protein and erythrocyte sedimentation rate?

A

hyperinflammation –> cytokines go to liver –> APPs and Ce-reactive protein and fibrinogen increase in production

48
Q

What would you test for if you suspected a complement or phagocytic cell deficit?

A

total complement assay

CBC+diff
assessment of respiratory burst = NBT test, flow cytometry, chemiluminecence
assessment of phagocytosis, chemotaxis, bactericidal activity

49
Q

In the first checkpoint of lymphocyte selection, what type of genetic rearrangements are selected for and saved from apoptosis?

A

in frame rearrangements that make a good protein

50
Q

Which immunoglobulin has the highest serum concentration and the longest half life?

A

IgG

51
Q

What do T helper 2 cells do?

A

release IL4 –> b cells proliferated to make Abs and kill extracellular microbes

52
Q

What complement induce B cells to go to lymph nodes?

A

C3d

53
Q

Where do granulomas develop?

A

in skin, GI tract, and genitourinary tract

54
Q

What types of infections are pts with CGD susceptible to?

Why?

A

recurrent pyogenic infection w/ catalase-positive organisms

in catalase-neg organisms, the H2O2 that is produced by all aerobic bacteria can be used to kill it in a phagosome; catalase + bacteria can inactivate H2O2 and pts with CGD have nothing else to kill them with

55
Q

What complements make up the MAC complex?

A

C5b, C6, C7, C8, C9

56
Q

What type of cell produces cathelicidins?

A

neutrophills

57
Q

What are the 3 professional APCs?

What type of MCH do they have?

A 
dendritic cells, macrophages, B cells
MHC class II
58
Q

What types of microbes are T helper 1 cells involved in?

A

intracellular

59
Q

What is LAD-I caused by?

A

mutations in gene for beta2 integrins (CD11/CD18) –> decreased levels on leukocytes

60
Q

What are the regions on a MHC that are most variable?

A

the parts that bind TCR and Ag = N terminus of alpha and beta

61
Q

What is the normal IL-23 and IL-17 axis?

A

microbes enter –> IL-23 increases –> neutrophils come in and kill microbes and apoptose –> phagocytosed by macrophages –> IL-23 decreases and downstream IL-17 decreases –> turn off inflammation

62
Q

What cells do CD8+ T cells kill?

A

any nucleated cell w/ MHC class I - Ag complex that is non-self

63
Q

What type of response do T-independent antigens induce in the body?

A

not a protein, so can’t break down for MHC

innate response and IgM antibodies produced

64
Q

What are the 5 types of possible primary immunodeficiencies?

A 
combined cellular and Ab = 15%
cellular = 5%
phagocytic cell = 10%
complement = 5%
antibody = 65%
65
Q

What do T helper 1 cells do?

A

release INF gamma –> activate macrophages to kill intracellular microbes and helps dev. of CD8 T cells

66
Q

What are the types of leukocyte adhesion deficiency?

A

LAD I
LAD II
LAD III

67
Q

What makes up the C5 convertase in the alternate pathway?

A

C3b + Bb + C3b

68
Q

How do cytotoxic T cells kill infected cells?

A

with perforins and granzymes like natural killer cells

69
Q

What happens to change a pro-B cell to a pre B cell?

A

heavy chain of BCR is finished recombining and forms a complete protein –> binds to surface w/ surrogate L chain

70
Q

What happens at the pre B cell stage?

A

check for intracellular signaling of heavy chain –> have a good heavy chain of BCR –> proliferate to make a lot of them

allelic exclusion of other genes for heavy chain

start VJC recombo of light chain

71
Q

What characterizes an immature B cell?

A

recombo of light and heavy chains are done and passed checkpoint by binding loosely to a self Ag

only contains IgM

Located in the bone marrow (then migrates to spleen)

72
Q

What is CD19?

A

cluster differentiating factor

found on mature T cells and B cells

73
Q

What is central tolerance?

A

checking receptor against self Ag

in BCR and TCR

74
Q

What is promiscuous gene expression?

A

when complete representation of self Ags must be present on mTECs to negatively select TCRs

75
Q

What is the sequence of B cell maturity from stem cell to mature B cell?

A

HSC –> pro b cell –> pre b cell –> immature b cell –> mature b cell

76
Q

What cell markers are on a pro b cell?

A

cd43
cd19
cd10

77
Q

What cell markers are on a pre b cell?

A

cd43

cd220

78
Q

What cell markers are on an immature b cell?

A

IgM

cd43

79
Q

What cytokine is the most important in getting neutrophils to cross the blood vessels and enter tissue?

A

IL-8

80
Q

What is the antibody made in a primary response?

what is the antibody made in a secondary response?

A

IgM = primary

IgG = secondary

81
Q

What CD is on RBCs?

A

CD47

82
Q

What CD is on mast cells?

A

CD23

83
Q

What CD is on scavenger cells?

A

CD36

84
Q

What CD is on monocytes?

A

CD14

85
Q

What CDs are on neutrophils?

A

CD15 and 16

86
Q

What CD is on dendritic cells?

A

CD80

HDM (Sophia's) (10 decks)

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