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STEP1 > Immuno > Flashcards

Immuno Flashcards

1
Q

medulla of lymph node is made up of medullary cords and sinuses. what types of cells are in the cords and sinuses? (2 each)

A

cords: lymphocytes and plasma cells
sinuses: reticular cells (type of fibroblasts) and macrophages

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2
Q

where are T cells in lymph node? (thus this part is underdeveloped in diGeorge syndrome)

A

paracortex

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3
Q

lung drains to ____ lymph nodes

A

hilar

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4
Q

trachea and esophagus drains to ____ lymph nodes

A

mediastinal

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5
Q

upper limb, breast, skin above umbilicus drains to ____ lymph nodes

A

axillary

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6
Q

colon from splenic flexure to upper rectum drains to ____ lymph nodes

A

inferior mesenteric

intestines above that -> superior mesenteric

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7
Q

liver, stomach, spleen, pancreas, upper duodenum drains to ____ lymph nodes

A

celiac

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8
Q

kidneys and utuerus drains to ____ lymph nodes

A

para-aortic (as do testis and ovaries)

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9
Q

lower rectum to anal canal above pectinate line, bladder, middle third of vagina, cervix, prostate drains to ____ lymph nodes

A

internal iliac

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10
Q

anal canal below pectinate line, skin below umbilicus, scrotum, vulva drains to ____ lymph nodes

A

superficial inguinal

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11
Q

dorsolateral foot, posterior calf drains to ____ lymph nodes

A

popliteal

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12
Q

lymph drainage pathway for right and left side of body

A

right side above diaphragm: R lymphatic duct -> junction of right sublcavian vein and IJVs

lower right and all of left: thoracic duct -> junction of left sublcavian and IJVs

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13
Q

spleen is protected by which ribs?

A

9-11. left side

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14
Q

where are T cells and B cells found in spleen?

A

T cells in pariarteriolar lymphatic sheath (PALS) in white pulp

B cells in follicles in white pulp

BOTH in WHITE pulp

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15
Q

molecluar/immune pathway by which splenic dysfunction or asplenia increases susceptibility to encapuslated organisms

A

decreased IgM -> decreaesd complement -> decreased C3b opsonization

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16
Q

4 things you would see in blood after splenectomy

A
  1. Howell-Jolly bodies
  2. target cells
  3. thrombocytosis (loss of sequestration and removal)
  4. lymphocytosis (loss of sequestration)
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17
Q

which mediastinum is thymus located in?

A

anterosuperior mediastinum

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18
Q

lymphocytes have what embryonic origin?

A

mesenchyme

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19
Q

MHC are encoded by which genes?

A

HLA

MHCI has one letter (HLA-A, HLA-B, HLA-C)
MHCII has 2 letters (HLA-DP, DQ, DR)

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20
Q

is MHC I or II 2 equal length chains?

A

II

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21
Q

is MHC I or II 2 one short and one long chain?

A

I

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22
Q

is MHC I or II has Beta2-microglobulin associated?

A

I

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23
Q

is MHC I or II has invariant chain associated?

A

II

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24
Q

perforin and granzymes are used by which immune cells?

A

NK cells and CD8 killer T cells

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25
Q

what happens in genetic deficiency of FOXP3?

A

that’s in T-reg cells. results in IPEX syndrome

Immune dysregulation
Polyendocrinopathy
Enteropathy
X-linked

+ skin and nail stuff (autoimmune derm stuff)

associated w/ diabetes in male infants!

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26
Q

what is the 2nd activating signal (for proliferation and surivival specifically) for CD4+ T cells? (first one is MHC binding obvis)

A

B7 on dendritic cell (APC) binds to CD28 on T cell

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27
Q

2nd activating signal for B cells

A

CD40 ligand on T cells, CD40 receptor on B cells. activates the T CELL, which then activates the B cell by IL-4 and IL-5

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28
Q

what is CD14

A

on macrophages. a co receptor for TLR4, recognizes LPS

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29
Q

TLR activation leads to upregulation of what nuclear transcription factor?

A

NF-kB

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30
Q

what are the 4 mediator/molecules that attract neutrophils?

A
  1. LTB4 leukotriene
  2. C5a
  3. IL-8
  4. bacterial products
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31
Q

what are the 4 mediator/molecules that attract neutrophils?

A
  1. LTB4 leukotriene
  2. C5a
  3. IL-8
  4. bacterial products
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32
Q

4 things that activate mast cells

A

tissue trauma
C3a
C5a
IgE cross link

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33
Q

histamines increases vascular permeability at 1._____ and vasodilates 2.______

A
  1. postcapillary venules (endothelial contraction)

2. arterioles

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34
Q

early and late phase mediators of mast cell activation

A

early: histamine
late: leukotrienes

note: this is diff from atopic asthma where early phase is mast cells so both histamine and leukotrienes, and late phase is eosinophils that release MBP

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35
Q

Kinin system is activated by what protein produced in liver?

A

Hageman factor aka Factor XII

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36
Q

bradykinin’s effect

A

same as histamine + pain

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37
Q

3 etiologies/pathogenesis of SCID and inheritance pattern

A
  1. cytokine receptor defect IL-2R gamma chain (needed for B and T cell activation) (X-linked)
  2. ADA deficiency (product buildup toxic to lymphocytes) (autosomal recessive)
  3. MHCII deficiency
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38
Q

x-linked/Bruton’s agammaglobulinemia

gene mutated? when does it present?

A

BTK gene = Bruton tyrosine kinase -> disordered B cell maturation ->agammaglobulinemia

6 months after birth (when maternal antibodies fade)

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39
Q

absent or scanty lymph nodes and tonsils, recurrent infections

A

brutons/x-linked agammaglobulinemia. BTK gene

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40
Q

CVID. presentation? pathogenesis?

A

defective B cell DIFFERENTIATION. (not maturation, like bruton’s) -> decreased plasma cells and immunoglobulins

increased sinus and enteric infections, presents later (2+ years) than bruton’s (6 months)

41
Q

hyper IgM syndrome is due to mutated ____ on T cells or ____ on B cells

inheritance pattern?

A

t cells CD40 ligand
B cells CD40 receptor

can’t activate the helper T cell -> no B cell activation -> no class switching. also failure to make germinal centers

x linked recessive

42
Q

kid has thrombocytopenia, recurrent infections, and eczema. what gene mutation does he have? inheritance pattern?

A

it’s wiskott aldrich syndrome

WASP gene(reorganize actin cytoskeletin -> antigen presentatino), X-linked recessive

43
Q

C1 esterase inhibitor deficiency has decreased levels of what complement mediator?

A

C4

44
Q

what part of antibody determines isotype (IgM, D, etc)

A

Fc region

45
Q

ROLLING:

involves ___ on endothelial cells, which bind to ___ on leukocytes

A

selectins on endothelial cells

sialyl Lewis X on leukocytes

46
Q

ADHESION: involves _____ on endothelium (upregulated by _______) and _______ on leukocytes (upregulated by _____)

A

TNF and IL-1 upregulate ICAM and VCAM on endothelium

C5a and LTB4 upregulate integrins on luekocytes

47
Q

Leukocyte adhesion deficiency has a defect in what protein on what cells?

A

LFA-1 integrin (CD18) protein on phagocytes

48
Q

which kinds of blood vessels do leukocytes transmigrate across?

A

postcap venules

49
Q

phagocytosis is enhanced by which Ig?

A

IgG (like C3b)

50
Q 
pt has: repeated infections
neutropenia
giant granules in leukocytes
defective primary hemostasis
albinism
peripheral neuropathy

dx? pathogenesis?

A

Chediak Higashi syndrome

all problems arise from defective lysosomal protein trafficking.

51
Q

in MPO deficiency, patients are mostly asympto but may have increased risk for _____ infections.

what color will nitroblue tetrazolium test show?

A

Candida

blue aka normal. b/c that’s for testing NADPH oxidase activity

NOTE: if absent cutaneous rxn to candida antigens, it’s actually chronic mucocutaneous candidiasis, where T cell dysfunction

52
Q

T cell receptor complex is made of TCR + _____

A

CD3

53
Q

cells and mediators mediating granuloma formation

A

macrophages MHCII and IL-12 -> CD4+ T cells -> Th1 subtype, secrete IFN-y -> convers macrophages into epithelioid histiocytes and giant cells

TNF-a from macrophages also induces and maintains

54
Q

features of Digeorge syndrome: CATCH22

A

Cardiac defects (conotruncal,tetralogy of Fallot)
Abnormal facies
Thymic hypo or aplasia (absent shadow on x ray)
Craniofacial stuff (bifid uvula, cleft palate)
Hypo PTH, hypo Ca2+ -> tetany
22q11 microsome deletion

55
Q

AIRE mutation results in what condition?

A

autoimmune polyendocrine syndrome

56
Q

Fas receptor is CD__

A

CD95

57
Q

Fas apoptosis pathway mutation results in what condition?

A

autoimmune lymphoproliferative syndrome (ALPS)

58
Q

how do T regs turn down immunie response?

A

release IL-10 and TGF-B (anti-inflammatory)

surface CTLA4 binds B7 on APC cells to block 2nd signal activation for CD4 helper cells

59
Q

thymus cortex or medulla where does positive and negative selection happen?

A

cortex: + selection

medulla : - selection

so maturation proceeds as cells move INWARD. hey it’s like androgen -> estrogen from theca cells to granulosa cells, also outward to in.

60
Q

HLA-B27 is associated w/ what autoimmune dzs? B a perfect(7) pair(2) PAIR

A

Psoriatic arthritis
Ankylosing spondylitis
IBD associated arthritis
Reactive arthritis

61
Q

deficiency of which complement proteins is associated w/ SLE?

A

the clearance onces. C1q, C4, C2

62
Q

clinical features of SLE: RASH OR PAIN

A

Rash (malar or discoid)
Arthritis (nonerosive)
Serositis (eg, pleuritis, pericarditis)
Hematologic disorders (eg, cytopenias)

Oral/nasopharyngeal ulcers (usually painless)
Renal disease

Photosensitivity
Antinuclear antibodies (sensitive)
Immunologic disorder (anti-dsDNA, anti-Sm) (specific)
Neurologic disorders (eg, seizures, psychosis)
63
Q

pretty much all the stuff in SLE is caused by IC deposition aka type III hypersensitivity rxn, except ________

A

hematologic. cytopenias due to antibodies against the blood cells

64
Q

antiphospholipid antibody is specifically directed against what?

A

the proteins bound to phospholipids. ex: anticardiolipin, anti-B2 glycoprotein I, lupus anticoagulant

65
Q

main feature of antiphospholipid antibody syndrome

A

hypercoagulable state (even tho falsely elevated PTT due to lupus anticoagulant antibody)

its what causes the thromboses -> pregnancy loss, DVT, stroke)

66
Q

______ antibody is present in drug induced lupus

A

antihistone antibody

67
Q

what type of hypersensitivty mediates Sjogren syndrome

A

type IV. lymphocyte mediated damage. (w/ fibrosis)

68
Q

antibodies present in Sjogren syndrome (3)

A

antinuclear antibodies,
rheumatoid factor (can be in the absence of rheumatoid arthritis),
antiribonucleoprotein antibodies: SS-A (anti-Ro) and/or SS-B (anti- La) (these can also be present in SLE)

69
Q

which antibody in Sjogren or SLE can cross placenta and cause neonatal lupus and congenital heart block?

A

Anti SSA (antiribonucleoprotein antibodies)

70
Q

pts with sjogren syndrome have increased risk for what kind of lymphoma?

A

B cell marginal cell lymphoma

71
Q

what molecular mediators in scleroderma?

A

endothelial dysfunction -> less NO, more endothelin -> inflammation
secretion of TGF-Beta and PDGF -> fibrosis

72
Q

immune dz that can cause pulmonary HTN

A

diffuse scleroderma (lung involvement can have interstitial fibrosis and pulm HTN)

73
Q

diffuse scleroderma has what antibodies?

A

anti Scl 70 = anti DNA topoisomerase I

74
Q

mixed connective tissue dz has what autoantibody?

A

anti U1 ribonucleprotein antibody

75
Q

naive mature B cells express which 2 Igs?

A

IgM, IgD

76
Q

IgA picks up secretory component from epithelial cells, which does what?

A

protects the Fc portion from luminal proteases

77
Q

CD55 deficiency -> _______

A

cd55 = decay accelerating factor = DAF

paroxysmal nocturnal hemoglobinuria

78
Q

what cytokine is released in response to IL-12 and increases MHC expression and antigen expression on all cells?

A

IFN-y

79
Q

CD marker for NK cells

A

CD56

80
Q

CD marker for hematopoietic stem cells

A

CD34

81
Q

what type of hypersensitivity rxn is hyperacute transplant rejection

A

type II

it’s bc of preformed antibodies to donor antigens

82
Q

what type of hypersensitivity rxn of polyarteritis nodosa

A

type III

83
Q

what type of hypersensitivity rxn is serum sickness and Arthurs rxn?

A

III

84
Q

what type of hypersensitivity rxn is contact dermatitis?

A

IV

85
Q

anti phospholipase A2 receptor

A

primary membranous nephropathy

86
Q

autosomal dominant hyper IgE syndrome is due to what gene mutation?

clinical presentation? FATED

A

STAT3 mutation -> Th17 deficiency -> decreased recruitment of neutrophils

Facies (coarse)
Abscesses (s. aureus, noninflamed)
Teeth (primary teeth retained)
Eosinophils and  IgE increased
Derm stuf (eczema)

bonus: bone fractures from minor trauma. IFN-y decreased

87
Q

Ataxia-telangiectasia has increased what serum marker?

decreased which Igs?

A

AFP

decreased IgA, IgG, IgE

88
Q

Ataxia-telangiectasia has increased risk of what?

A

lymphoma and leukemia (but they also have lymphopenia!)

89
Q

pt comes in w/ spider angiomas and IgA deficiency. what other thing do they have? what is the genetic basis?

A

cerebellar atrophy w/ ataxia. this is ataxia-telangiectasia. ATM gene defect -> can’t detect DNA damage -> cell cycle proceeds -> mutations accumulate

90
Q

which Ig’s are elevated in Wiskott Aldrich syndrome?

A

IgA, IgE

91
Q

inheritance pattern of CGD? which tests are abnormal?

A

X linked recessive

dihydrorhodamine (flow cytometry) test = decreased green fluorescence
nitroblue tetrazolium dye reduction test doesn’t turn blue

92
Q

mediators of acute transplant rejection and presentation? hypersensitivity type?

A

IV: CD8 T cells against donor MHCs
II: antibodies/humoral

presents as vasculitis of graft vessels w/ dense interstitial lymphocytic infiltrate

93
Q

mediators of chronic transplant rejection and presentation? hypersensitivity type?

A

IV and II: CD4 T cells activated by recipient APCs presenting donor peptides (like MHC)

cytokines -> proliferation of vascular smooth muscle, parenchymal atrophy, interstitial  fibrosis. Dominated by arteriosclerosis.
Organ-specific examples:
ƒ Bronchiolitis obliterans (lung)
ƒ Accelerated atherosclerosis
(heart)
ƒ Chronic graft nephropathy
(kidney)
ƒ Vanishing bile duct syndrome
(liver)
94
Q

graft vs host dz presentation?

A

Maculopapular rash, jaundice, diarrhea, hepatosplenomegaly. severe organ dysfunction.
GI/rectal infiltrate of lymphocytes

95
Q

transmigration/diapedesis of WBCs involves ___ on leukocyte and ____ on vessel

A

PECAM (CD31) on both!

96
Q

Hageman factor is important mediator in DIC from gram negative sepsis. what 3 pathways does it activate?

A
  1. coagulation and fibrinolytic systems
  2. complement
  3. Kinin system
97
Q

Fc receptor on cells i CD___

A

CD16

98
Q

how do you know that neutrophils are immature? like if there’s a lot of them a left shift has occured?

A

decreased Fc receptors (CD16)

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