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FA > immuno > Flashcards

immuno Flashcards

1
Q

secondary lymphoid organ with many afferents, one or more efferents

A

lymph node

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2
Q

lymph node features

A

encapsulated with trabeculae

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3
Q

LN functions

A

nonspecific filtration by macrophages, storage and activation of B and T cells, antibody production

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4
Q

follicle

A

located in outer cortex

B cell localization and proliferation

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5
Q

primary follicle

A

dense and dormant

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6
Q

secondary follicle

A

pale germinal centers - ACTIVE

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7
Q

medulla is made up of

A

medullary cords and medullary sinuses

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8
Q

medullary cords

A

closely packed lymphocytes and PLASMA cells

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9
Q

medullary sinuses

A

communicate with efferent lymphatics

contain reticular cells and MACROPHAGES

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10
Q

paracortex

A

houses T cells
between cortex and medulla
high endothelial (post capillary) venules - T + B cells enter
digeorges - poorly develped

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11
Q

extreme cellular immune response

A

paracortex greatly enlarges EX: viral

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12
Q

where do UL and lateral breast drain

A

axillary LN

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13
Q

where does stomach drain

A

celiac LN

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14
Q

where do duodeneum and jejunum drain

A

superior mesenteric LN

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15
Q

where does sigmoid colon drain

A

colic –> inf mesenteric LN

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16
Q

where does rectum drain (above pectinate line)

A

internal iliac LN

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17
Q

where does anal canal drain (below pectinate line)

A

superficial inguinal LN

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18
Q

where do tests drain

A

superficial and deep plexuses –> para-aortic LN

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19
Q

where does scrotum drain

A

superficial inguinal LN

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20
Q

where does superficial thigh drain

A

superficial inguinal LN

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21
Q

where does lateral side of dorsum of foot drain

A

popliteal LN

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22
Q

right lymphatic duct

A

right arm, right chest, right half of head

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23
Q

thoracic duct

A

everything else

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24
Q

sinusoides of spleen

A

long vasc channel in red pulp

fenestrated barrel hoop basement membrane

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25
Q

where are T cells found in the spleen

A

white pulp - periarterial lymphatic sheath (PALS)

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26
Q

where are the B cells found in the spleen

A

white pulp - follicles

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27
Q

spleen function

A

macrophages remove encapsulated bacteria

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28
Q

splenic dysfunction

A

less IgM –> less complement activatoin –> less C3b opsonization –> increase susceptibility to encap organsisms

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29
Q

encap organisms

A

SHINe SKiS

step pneumo, h flu type B, n meningitis, salmonella, klebsiella, group B strep

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30
Q

what is seen postsplenectomy

A

nuclear remnants = howell-jolly bodies
target cells
thrombocytosis

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31
Q

spleen germinal center

A

B cells

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32
Q

spleen PALS

A

T cells

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33
Q

spleen marginal zone

A

APCs

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34
Q

Spleen red pulp

A

RBC

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35
Q

site of T cell differentiation and maturation

A

thymus

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36
Q

thymus

A

3rd branchial pouch - epithelium

lymphocytes - mesenchymal origin

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37
Q

thymic cortex

A

dense - immature T cells

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38
Q

thymuc medulla

A

pale - mature T cells + epithelial reticular cells with Hassall’s corpuscles

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39
Q

where does positive selection occur

A

cortex - MHC restriction

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40
Q

where does negative selection occur

A

medulla - nonreactive to self

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41
Q

germinline coded, fast and nonspecific response, no memory

A

innate immunity - neutrophils, macrophages, denderitic cells, NK cells and complement

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42
Q

VDJ recombination, slow on first exposure with faster in memory response

A

adaptive immunity - B and T cells and circulating antibody

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43
Q

MHC

A

encoded by HLA genes

present antigen fragments to T cells and bind TCR

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44
Q

HLA-A, HLA-B, HLA-C

A

MHC I

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45
Q

MHC 1

A

binds TCR + CD8

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46
Q

mediates viral immunity

A

MHC1

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47
Q

binds with B2 microglobulin to transport to cell surface

A

MHC 1

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48
Q

HLA-DR, HLA-DP, HLA-DQ

A

MHC 2

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49
Q

MHC 2

A

binds TCR + CD4

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50
Q

where are MHC class 1 expressed

A

all nucleated cells (not rbc)

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51
Q

where are MHC class 2 expressed

A

only on APC

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52
Q

where is MHC class 1 antigen loaded

A

in RER with intracellular peptides

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53
Q

where is MHC class 2 antigen loaded

A

following release of invariant chain in an acidified endosome

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54
Q

HLA A3

A

hemochromatosis

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55
Q

HLA B27

A

PAIR - psoriasis, ankylosing spondylitis, IBD, reiters syndrome

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56
Q

HLA DQ2/DQ8

A

celiacs

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57
Q

HLA DR2

A

MS, hay fever, SLE, goodpastures

SHaGgy M

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58
Q

HLA DR3

A

DM1, graves

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59
Q

HLA DR4

A

RA, DM1

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60
Q

HLA DR5

A

PA –> B12 deficient, hashimotos

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61
Q

only lymphocyte member of innate immune system

A

NK cells

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62
Q

NK cells

A

use perforin and granzymes to induce apoptosis of virally infection cells and tumor cells

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63
Q

what enhances NK activity

A

IL-2, IL-12, IFN-alpha, IFN-beta

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64
Q

when are NK cells induced to kill

A

exposed to nonspecific activation signal on target cell/ absence of class I MHC on target cell surface

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65
Q

B cell function

A

make antibody - opsonize bacteria

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66
Q

which B cells neutralize viruses

A

IgG

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67
Q

which B cells activate complement

A

IgG and IgM

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68
Q

which B cells sensitize mast cells

A

IgE

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69
Q

Cell seen in allergies

A

B cell = IgE - HS I

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70
Q

Cell seen in cytotoxic rxn

A

B cell = IgG - HS II

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71
Q

Cell seen in IC rxn

A

B cell = IgG - HS III

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72
Q

hyperacute and humorally mediated acute and chronic organ rejection

A

B cells

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73
Q

CD4 T cell function

A

help make B cell antibody + produce cytokines to active other cells

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74
Q

CD8 T cell function

A

kill virus-infected cells directly

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75
Q

which cell is seen in delayed cell-mediated HS (IV)

A

T cells

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76
Q

which tell is seen in acute and chronic cellular organ rejection

A

T cells

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77
Q

what selection happens in thymic medulla

A

negative selection - T cells expressing TCRs with HIGH AFFINITY for self antigens –> apoptososis

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78
Q

what selection happens in thymic cortex

A

positive selection - T cells expressing TCRs capable of binding surface self MHC molecules survive

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79
Q

what do cytotoxic T cells do in thymus

A

CD8+ kill virus-infected cells, neoplastic cells and donor graft cells

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80
Q

what stimulates CD4 –> Th1

A

IL-12

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81
Q

what stimulates CD4–> Th2

A

IL-4

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82
Q

where do Th1 and Th2 develop

A

LN

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83
Q

only APC that can activate naiive T cell

A

dendritic cell

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84
Q

APC’s

A

DMB

denderitic cells, mo, B cells

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85
Q

two signals for naiive T cell activation

A

dendritic cell phagocytosis

  1. ag. presented on MHC II/I + recognized by TCR on Th/Tc
  2. costim signal = CD28 (naiive T cell) + B7 (dendritic cell)
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86
Q

B cell activation

A 
B cell R-medated endocytosis
1. ag. presented on MHC II - Th
2. costim signal = CD40 (B cell) + CD40 L (Th) 
B cella ctivates --> class switching, affinity maturation, ab. production
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87
Q

B cell class switching

A

Th –> cytokines –> Ig class switching of B cell

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88
Q

what do Th1 cells secrete

A

IFN-y

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89
Q

what do Th2 cells secrete

A

IL-4,5,10,13

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90
Q

Th1 cell function

A

activate macrophages

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91
Q

Th2 cell function

A

recruit eosinophils for parasite defense + promote IgE production by B cells

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92
Q

what inhibits Th1

A

IL-4 and IL-10 (from Th2)

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93
Q

what inhibits Th2

A

IFN-y (from Th1)

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94
Q

macrophage-lymphocyte interaction

A

active lymphocytes (release IFN-y) and mo (release IL1, TNF-alpha) stimulate one another

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95
Q

cytotoxic t cell MOA

A

induce apoptosis –> kill virus-infected cells, neoplastic cells + donor graft cells
release cytotoxic granules containing preformed proteins (perforin, granzyme, and granulysin)

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96
Q

perforin

A

deliver content of granules into target cell

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97
Q

granzyme

A

serine protease, activates apoptosis inside target cell

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98
Q

granulysin

A

antimicrobial, induces apoptosis

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99
Q

cytotoxic T cell general

A

have CD8 –> bind MHC 1 on virus-infected cells

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100
Q

regulatory T cells

A

suppress CD4 + CD* T cell effector function –> help maintain specific immune tolerance

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101
Q

what do regulatory T cells

A

CD 3, 4, 25 surface markers

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102
Q

CD 25

A

alpha chain of IL-2 receptor

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103
Q

what do activated regulatory T cells produce

A

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

Tay lives in apt 10B and is antisocial

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104
Q

antibody: variable part L and H chain

A

recogznie antigens

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105
Q

antibody: Fc portion IgM and IgG

A

fixes complement

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106
Q

antibody: heavy chain

A

contributes to Fc and Fab fractions

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107
Q

antibody: light chain

A

contributes only to Fab fraction

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108
Q

Fab

A

antigen binding fragment

determines idiotype - only 1 antigenic specificity expressed per B cell (unique antigen-binding pocket)

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109
Q

Fc

A

constant, carboxy terminal, complement binding at Ch2 (IgG and IgM), carbohydrate side chaind
determines isotype - IgM, IgD, etc.

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110
Q

ab diversity

A

random recomb of VJ (light chain) or V(D)J (heavy chain genes, random comb heavy chains w/ light chains, somatic hypermutation (following ag stimulation), addition of nucleotides to DNA during recomb by terminal deoxynucleotidyl transferase

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111
Q

Fc Ch2

A

complement binding

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112
Q

Fc Ch3

A

macrophage binding

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113
Q

Fab contains

A

VJD, Ch1, Cl

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114
Q

opsonization

A

anitbody promotes phagocytosis

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115
Q

neutralization

A

antibody prevents bacterial adherence

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116
Q

complement activation

A

MAC - C3b

antibody activates complement, enhancing opsonization and lysis

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117
Q

immunoglobulin isotype

A

mature B lymphocytes express IgM and IgD –> class switching (cytokines + CD40 mediate gene rearrangement) –>plasma cells - secrete IgA, IgE, IgG

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118
Q

IgG

A

secondary delayed response to ag

fixes complement, crosses placenta, opsonize bact, neutralize bact toxins + viruses

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119
Q

IgA

A

prevents bact/virus attachment to MUCOUS MEMBRANES; doesnt fix complement
circulation - monomer
secreted - dimer
crosses epithelial cells via trancytosis
picks up secretory componenet from epithelial cells before secretion

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120
Q

IgM

A

primary immediate response to antigen; fixes complement
ag R = surface of B cells (monomer)
pentamer - trap free ag out of tissue while humoral response evolves

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121
Q

IgD

A

B cells and serum

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122
Q

IgE

A

binds mast cells and basophils
exposed to allergen –> cross-links –> mediates type I HS via release of inflam mediators (histamine)
activates eosinophils –> mediates immunity to worms

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123
Q

most abundant immunoglobulin isotype

A

IgG

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124
Q

which mmunoglobulin isotype provides infants with passive immunity

A

IgG

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125
Q

which immunoglobulin isotype is found in secretions and colostrum

A

IgA
secretions = tears, saliva, mucus
colostrum = early breast milk

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126
Q

which immunoglobulin isotype has lowest concentration in the serum

A

IgE

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127
Q

thymus-independent antigens

A

stimulate release of antibodies (no memory)

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128
Q

thymus-dependent antigens

A 
contain protein component (dipth vaccine)
direct contact of B cells with Th cells (CD40-40L) --> class switching + immunologic memory
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129
Q

what type of immunity does complement play a role in?

A

innate immunity (and inflammation)

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130
Q

what does MAC in complement do?

A

help fight against gram negative bacteria

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131
Q

classic complement activation

A

IgM and IgG

classic GM cars

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132
Q

alternative complement activation

A

microbe surface molecules

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133
Q

lectin complement activation

A

mannose/sugars on microbe

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134
Q

complement that binds bacteria

A

C3b

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135
Q

complement responsible for anaphylaxis

A

C3a + C5a

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136
Q

neutrophil chemotaxis complement

A

C5a

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137
Q

cytolysis by MAC complement

A

C5b-9

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138
Q

primary opsonins in bact defense

A

C3b and IgG

139
Q

helps clear immune complexes

140
Q

what prevents complement activation on self cells (RBC)

A

DAF (GPI anchored enzyme) and C1 esterase inhibitor

141
Q

which complement pathway is innate and does not require antibody

A

alternative pathway

142
Q

C1 esterase inhibitor deficiency

A

hereditary angioedema

143
Q

whats CI in C1 esterase inhibitor deficiency

A

ACE inhibitors

144
Q

C3 deficiency

A

severe recurrent pyogenic sinus + RT infections

increase susceptibility to type 3 HS rxns

145
Q

C5-9 deficiency

146
Q

DAF deficiency

A

complement-mediated lysis of RBC + PNH

147
Q

cytokines secreted by macrophages

A

IL-1, 6, 8, 12,

TNF-alpha

148
Q

cytokines secreted by all T cells

A

IL-2 and IL-3

149
Q

cytokines secretes from Th1 cells

150
Q

cytokines secreted from Th2 cells

A

IL-4, 5, 10, 13

151
Q

IL-1

A

fever (hot)
activates endothelium –> express adhesion molecules
chemokine secretion –> recruit leukocytes
endogenous pyrogen –> fever, acute inflammation
ACE

152
Q

IL-2

A

stimulates T cells (helper, cyto + reg)

153
Q

IL-3

A

stimulates bone marrow SC (functions like GM-CSF)

154
Q

IL-4

A

stimulates IgE production
differentiation into Th2 cells
growth of B cells
class switching –> IgE and IgG

155
Q

IL-5

A

stimulate IgA production
differentiation of B cells
eosinophils!

156
Q

IL-6

A

endogenous pyrogen
also secreted by Th2 cells
fever + acute-phase proteins

157
Q

IL-8

A

major chemotactic factor neutrophils

clean up aisle 8 - neutrophilcs recruited by IL-8 to clear infections

158
Q

IL-10

A

moadulates inflamm response
also secreted by reg T cells
inhibits T cells and Th1

159
Q

IL-12

A

T cells –> Th1
actives NK cells
also secreted by B cells

160
Q

TNF-alpha

A

SEPTIC SHOCK
activates endothelium
leukocyte recruitment and vascular leak

161
Q

IFN-gamma

A

activates macrophages and Th1 cells
suppreses Th2 cells
antiviral and antitumor properties

162
Q

TGF beta

A

similar to IL-10 - inhibits inflammation

163
Q

interferons

A

interFERES with viruses
proteins that place uninfected cells in an antiviral state
induce production of ribonuclease –> degrades VIRAL (not host) mRNA –> inhibits viral protein synthesis

164
Q

interferon alpha and beta

A

inhibit viral protein synthesis

165
Q

interferon gamma

A

increase MHC I and II expression and antigen presentation in all cells

166
Q

activates NK cells to kill virus-infected cells

A

interferons

167
Q

all cells have MHC I except

168
Q

TCR

A

binds antigen-MHC complex

169
Q

CD3

A

TCR signal transduction

170
Q

CD28

A

on naiive T cells binds B7 on APC

171
Q

helper T cells

A

CD4, CD40L

172
Q

cytotoxic T cells

173
Q

Ig

A

B cell that binds antigen

174
Q

B cells

A

CD 19, 20, 21, 40
MHC II
B7

175
Q

receptors for EBV

176
Q

macrophages

A

CD 14, 40

MHC II, B7

177
Q

enhanced phagocytosis of macrophages

A

Fc and C3b receptors

178
Q

NK cells

A

CD 16 - binds Fc of IgG

Cd 56 - unique NK marker

179
Q

anergy

A

self-reactive T cell become nonreactive without costim molecule
less complete tolerance in B cells

180
Q

superantigens

A 
strep pyogenes + staph aureus
cross link Beta region of TCR to MHC class II on APCs --> can activate any T cell --> massive release of cytokines
181
Q

endotoxins/LPS

A

gram negative: bind to endotoxin receptor CD14 –> directly stimulate mo (Th cells not involved)

182
Q

antigen variation mechanisms

A

DNA rearrangement and RNA segment reassortment (flu major shift)

183
Q

antigen variation examples bact

A

salmonella - 2 flagellar variants
boreelia - relapsing fever
n. gonorrhea - pilus protein

184
Q

antigen variation examples virus

A

flu (major = shift; minor = drift)

185
Q

antigen variation examples parasites

A

trypanosomes - programmed rearrangement

186
Q

passive immunity means of acquisition

A

recieve preformed antibodies

187
Q

active immunity means of acquisition

A

exposure to foreign antigens

188
Q

passive immunity onset

189
Q

active immunity onset

190
Q

passive immunity duration

A

short span of antibodies (t 1/2 = 3 weeks)

191
Q

active immunity duration

A

long-lasting protection (memory)

192
Q

passive immunity EX

A

IgA in breast milk, antitoxin, humanized monoclonal antibody

193
Q

active immunity EX

A

natural inffection, vaccines, toxoid

194
Q

when can combined passive and active immunizations be given

A

hep B or rabies exposure

195
Q

when are patients given preformed antibodies and what kind of immunity is this

A

passive immunity
to be healed rapidly
tetanus toxin, botulinum toxin, HBV, rabies virus

196
Q

Type 1 HS

A

atopic + anaphylatic - fast and first

cross-links IgE on mast cells and basophils –> release of histamine *rapid after ag exposure due to preformed antibody

197
Q

Type 2 HS

A

cytotoxic
IgG and IgM –> ag on enemy cell –> cellular destruction
antibody + complement –> MAC
dz specific to tissue ag found

198
Q

Type 3 HS

A

immune complex mediated - antigen-antibody (IgG)-complement –> neutrophils –> lysosomal enzymes

199
Q

Type 4 HS

A

delayed HS - t cell mediated
sens T lymphocytes + ag –> release lymphokids –> mo activation
4 Ts = T lymphocytes, transplant rejection, TB skin tests, touching (contact dermatitis)

200
Q

antibody mediated HS reactions

A

HS I, II, III

201
Q

Type 1 HS test

A

skin test - specific IgE

202
Q

Type 2 HS mechanisms

A

opsonization - phagocytosis/complement activation
complemented mediated lysis
ADCC - NK cells

203
Q

Type 2 HS test

A

coombs (direct and indirect)

204
Q

serum sickness

A

type III HS
ab to foreign produced within 5 days –> IC form + deposited in membranes –> fix complement –> tissue damage
most now caused by drugs actin as haptens
sx - fever, urticaria, arthralgias, proteinuria, lymphademopathy 5-10 d post-ag exposure
FLAP U

205
Q

arthrus reaction

A

Type III HS
local subacute
intradermal injection of ag –> antibodies –> ag-antibody complexes
CANE - complement activation, necrosis, edema
cause = antigen-antibody complexes

206
Q

type III HS test

A

immunofluorescent staining

207
Q

type IV HS test

A

patch test, PPD

208
Q

type 3 examples

A

apples - arthrus rxn, polyarteritis nodosa, poststrep glomerulonephritis, sle, serum sickness
vasculitis + systemic manifestation

209
Q

type 4 examples

A

can you C GGraMP - contact dermatitis, GBS, GVHD, MS, PPD

210
Q

blood transfusion rxn: allergic

A

HS I vs plasma proteins in transfused blood –> urticaria, prurius, wheezing, fever

211
Q

blood transfusion rxn: anaphylactic

A

severeeee *IgA-deficient pts must recieve blood that lacks IgA –> dyspnea, bronchospasm, hypotension, resp arrest, shock

212
Q

febrile nonhemolytic transfusion reaction

A

HS II *host ab. vs donor HLA ag + leukocytes –> fever, headaches, chills, flushing

213
Q

acute hemolytic transfusion reaction

A

HS II *intravasc hemolysis (ABO) or extravasc hemolysis (host ab. vs foreign ag or donor RBC) –> fever, hypotension, tachypena, tachycardia, flank pain, hemoglobinemia (intravasc), jaundice (extravasc)

214
Q

allergic blood transfusion rxn tx

A

antihistamines

215
Q

antinuclear antibodies

A

SLE nonspecific

216
Q

anti-dsDNA

217
Q

anti-Smith

218
Q

antihistone

A

drug-induced lupus

219
Q

rheumatoid factor

220
Q

anti-CCP

221
Q

anticentromere

A

scleroderma - CREST syndromes

222
Q

anti-Scl-70

A

scleroderma diffuse

anti-DNA topoisomerase I

223
Q

antimitochondrial

A

primary biliary cirrhosis

224
Q

IgA antiendomysial

225
Q

IgA anti-tissue transglutaminase

226
Q

anti-basement membrane

A

goodpasture

227
Q

anti-desmoglein

A

pemphigus vulgaris

228
Q

antimicrosomal

A

hashimotos thyroiditis

229
Q

antithyroglobulin

A

hashimotos

230
Q

anti-jo-1

A

polymyositis, dermatomyositis

231
Q

anti-SRP

A

polymyositis, dermatomyositis

232
Q

anti-Mi-2

A

polymyositis, dermatomyositis

233
Q

anti-SSA

A

sjogrens syndrome

anti-Ro

234
Q

anti-SSB

A

sjogrens syndrome

anti-La

235
Q

anti-U1 RNP

A

mixed CT disease

ribonucleotide protein

236
Q

anti-smooth muscle

A

autoimmune hepatitits

237
Q

anti-glutamamte decarboxylase

238
Q

c-ANCA

A

granulomatosis w/ polyangiitis (wegeners)

239
Q

p-ANCA

A

microscopic polyangitis, churg-strauss

240
Q

bacterial infections in pt w/ no T cells

241
Q

bacterial infections in pt w/ no B cells

A

encapsules - SHiN SKis

strep pneumo, h flu type b, n meningitis, salmonella, klebsiella, group b strep

242
Q

bacterial infections in pt w/ no granulocyte

A

staph, burkholderia cepacia, serratia, nocardia

243
Q

bacterial infections in pt w/ no complement

A

neisseria (no MAC)

244
Q

viral infection in pt w/ no T cells

A

CMV, EBV, VZV, chronic infection w/ resp/GI viruses

245
Q

viral infections in pt w/ no B cells

A

enteroviral encephalitis, polio virus

CI = live vaccine

246
Q

fungi/parasitic infection in pt w/ no T cells

A

candida, PCP

247
Q

fungi/parasitic infection in pt w/ no B cells

A

GI giardiasis (no IgA)

248
Q

fungi/parasitic infection in pt w/ no granulocyte

A

candidia, aspergillus

249
Q

B cell deficiencies tend to produce

A

recurrent bacteiral infections

250
Q

T cell deficiencies tend to produce

A

fungal and viral infections

251
Q

X linked agammaglobulinemia AKA

252
Q

X linked agammaglobulinemia defect

A

X-linked recessive - increase in boys

defect in BTK (tyrosine kinase gene) –> no B cell maturations

253
Q

X linked agammaglobulinemia sx

A

opsonization defect –> recurr bact infections after 6 months (low maternal IgG)

254
Q

X linked agammaglobulinemia dx

A

decrease maturation, number of B cells, immunoglobulins

255
Q

most common primary immunodeficiency

A

Selective IgA deficiency

256
Q

Selective IgA deficiency sx

A

asx
sinopulmonary infections, GI infections, autoimmune disease
Anaphylaxis to IgA-containing blood products

257
Q

Selective IgA deficiency dx

A

IgA < 7 mg/dL

heterophile antibody –> false-positive beta-HCG

258
Q

CVID

A

common variable immunodeficiency

259
Q

CVID defect

A

defect in B cell maturation - many cuases

260
Q

CVID sx

A

20-30s; increase risk autoimmune dz, lymphoma, sinopulmonary infections

261
Q

CVID dx

A

normal number B cells but DECREASED PLASMA CELLS AND IMMUNOGLOBULINS

262
Q

Thymic aplasia

A

digeorges syndrome

263
Q

digeorges syndrome defect

A

22q11 deletion

failure to develop 3/4th pharyngeal pouches

264
Q

digeorges syndrome sx

A

hypocalcemia –> tetany
T cell deficinecy –> recurrent virla/fungal infections
congenital heart and great vessel defects

265
Q

digeorges syndrome dx

A

thymus and parathyroid cells fail to develop –> decrease T cells, PTH, Ca2+

266
Q

absent thymic shadow on CXR

A

digeorges syndrome

267
Q

IL-12 R deficiency defect

A

decrease Th1 response
REMEMBER: Th –> IL-12 –> Th1
Th –> IL-4 –> Th2

268
Q

IL-12 R deficiency sx

A

disseminated mycobacterial infections

269
Q

IL-12 R deficiency dx

A

low IFN-gamma (from Th1)

270
Q

hyper IgE syndrome AKA

A

jobs syndrome

271
Q

jobs syndrome defect

A

Th1 cells fail to produce IFN-y –> neutrophils cant respond to chemotactic stimuli

272
Q

jobs syndrome sx

A

FATED

course facies, cold (noninflamted) staph abscesses, retains primary teeth, high IgE, dermatologic problems (eczema)

273
Q

jobs syndrome dx

274
Q

chronic mucocutaneous candidiasis defect

A

T cell dysfunction

275
Q

chronic mucocutaneous candidiasis sx

A

candidiana albicans infection of skin and mucous membranes

276
Q

SCID defect

A

most common x-linked = defective IL-2

adenosine deaminase deficiency

277
Q

SCID sx

A

failure to thrive, chronic diarrhea, thrush

reccurent infections

278
Q

SCID dx

A 
decreased t-cell recombinant excision circles
absent thymic shadow
LN biopsy - germinal centers
peripheral blood smears - B cells
flow cytometry - T cells
279
Q

ataxia-telangiectasia defect

A

defect in ATM gene - codes for DNA repair enzymes

280
Q

ataxia-telangiectasia sx

A

TRIAD: cerebellar defects (ataxia), spider angiomas (telangiectasia), IgA deficiency

281
Q

ataxia-telangiectasia dx

A

increase AFP

282
Q

hyper IgM syndrome defect

A

defective CD40L on Th = unable to class switch

283
Q

hyper IgM syndrome sx

A

severe pyogenic infections early in life

284
Q

hyper IgM syndrome dx

A

high IgM

LOWWWW IgG, IgA, IgE

285
Q

wiskott-aldrich syndrome defect

A

x-linked

WASP gene on X chromosome –> T cells unable to reorganize actin cytoskeleton

286
Q

wiskott-aldrich syndrome sx

A

TRIAD TIE = thrombocytopenia purpura, infections, eczema

287
Q

wiskott-aldrich syndrome dx

A

high IgE, IgA
low IgM
thrombocytopenia

288
Q

leukocyte adhesion deficiency (type 1) defect

A

LFA-1 integrin (CD18) protein on phagocytes

289
Q

leukocyte adhesion deficiency (type 1) sx

A

recurrent bacterial infections, absent pus formation, delayed separation of umbilical cord

290
Q

leukocyte adhesion deficiency (type 1) dx

A

neutrophilia

291
Q

chediak-higashi syndrome defect

A

AR defect in LYST

microtubule dysfunction in phagosome-lysosome fusion

292
Q

chediak-higashi syndrome sx

A

recurrent pyogenic infections by staph + strep

partial albinism, peripheral neuropathy

293
Q

chediak-higashi syndrome dx

A

giant granules in neutrophils

294
Q

chronic granulomatous disease defect

A

lack NADPH oxidase –> decrease ROS (superoxide) + absent resp burst in neutrophils

295
Q

chronic granulomatous disease sx

A

increase susceptibility to catalase positive organisms (stapg, e coli, aspergillus)

296
Q

chronic granulomatous disease dx

A

abnormal dihydrorhodamine (DHR) flow cymotry test

297
Q

autograft

298
Q

syngeneic graft

A

from identical twin or clone

299
Q

allograft

A

from nonidentical individual of same species

300
Q

xenograft

A

from different species

301
Q

hyperacute rejection onset

302
Q

hyperacute rejection pathogenesis

A 
ab mediates (type 2)
preformed anti-donor ab in transplant recipient
303
Q

hyperacute rejection features

A

occludes graft vessels –>ischemia and necrosis

304
Q

acute rejection onset

305
Q

acute rejection pathogenesis

A

cell mediated
CTLs reacting vs foreign MHCs
reversible w/ immmunosuppresants (cyclopsporine, muromonab-DC3)

306
Q

acute rejection features

A

vasculitis of graft vessels w/ dense interstitial lymphocytic infiltrate

307
Q

chronic rejection onset

A

months-years

308
Q

chronic rejection pathogenesis

A

class I-MHC non self percieved by CTLs as class I-MHCself presenting on non-self antigen

309
Q

chronic rejection features

A

irreversible
t cell and ab.-mediated vascular damage (obliterative vascular fibrosis)
fibrosis of graft tissue and blood vessels

310
Q

GVHD pathogenesis

A

grafted immunocompetent T cells in IC disease host –> reject cells with foreign proteins –> severe organ dysfunction

311
Q

GVHD features

A

maculopapular rash, jaundice, hepatosplenomegaly, diarrhea

BM + Liver transplant (rich in lymphocytes)

312
Q

potentially beneficial in BM transplant

A

GVHD –> to kill cancer cells!

313
Q

cyclosporins mechanism

A

binds cyclophilins –> inhibits calcineurin –> prevents IL-2 and R production –> inhibits differentiation/activation of T cells

314
Q

cyclosporins clinical use

A

suppresses organ rejection post-transplant; some autoimmune dz

315
Q

cyclosporins toxicity

A

nephrotoxicity, HTN, hyperlipidemia, hyperglycemia, tremor, gingival hyperplasia, hirsuitism
HHHunTiNG

316
Q

tacrolimus (FK-506) mechanism

A

binds FK-bidning protein –> inhibits calcineurin + IL-2 secretion

317
Q

tacrolimus clinical use

A

potent immunosuppresive used in organ transplant recipients

318
Q

tacrolimus toxicity

A

nephrotoxicity, HTN, hyperlipidemia, hyperglycemia, tremor

HHunTiN

319
Q

azathioprine mechanism

A

6-mercaptopurine antimetabolite precursor interfers w/ nucleic acid metabolism/synthesis –> toxic to proliferating lymphocytes

320
Q

azathioprine clincial use

A

kdiney transplant,a utoimmune dz (glomerulonephritis + hemolytic anemia)

321
Q

azathioprine toxicity

A

BM suppression

active metabolite metabolized by xanthine oxidase –> increased allopurinol = toxic effects

322
Q

muromonab-CD3 (OKT3) mechanism

A

monoclonal antibody binds CD3 (epsilon chain) on T cell surface –> block cell interaction w/ CD3 protein responsible for T-cell signal transduction

323
Q

muromonab-CD3 (OKT3) clinical use

A

immunosuppresion post-kidney transplant

324
Q

muromonab-CD3 (OKT3) toxicity

A

cytokine release syndrome, HS ran

325
Q

sirolimus (rapamycin) mechanism

A

inhibits mTOR –> inhibits T cell prolif in response to IL-2

326
Q

sirolimus (rapamycin) clinical use

A

immunosuppresion post-kidney transplant w/ cyclopsporine + corticostoeroids; also used w/ drug-eluting stents

327
Q

sirolimus (rapamycin) toxicity

A

hyperlipidemia, thrombocytopenia, leukopenia

328
Q

aldesleukin used for

A

renal cell carcinoma, metastiac melanoma

329
Q

epoetin alfa used for

A

anemias (renal fialure)

330
Q

filgastrim used for

A

recover BM

331
Q

sargramostim used for

A

recover BM

332
Q

alpha-interferon used for

A

hep B + C, kaposis sarcoma, leukemias, malignant melanoma

333
Q

beta-interferon used for

334
Q

gamma-interferon used for

335
Q

oprelvekin used for

A

thrombocytopenia

336
Q

thrombopoietin used for

A

thrombocytopenia

337
Q

muromonab-CD3

A

CD3 –> prevents acute transplant rejection

338
Q

Digoxin Immune Fab

A

digoxin –> antidote for digoxin intoxication

339
Q

infliximab

A

TNF-alpha –> CD, RA< psoriatic arthritis, akylosing spondylitis

340
Q

adalimumab

A

TNF-alpha –> CD, RA, psoriatic arthritis

341
Q

abciximab

A

glcyoprotein IIb/IIIa –> prevent cardiac ischemia in unstable angina + pts treated w/ PCI

342
Q

trastuzumab

A

HER2 –> HER-2 overexpressing breast cance

343
Q

rituximab

A

CD20 –> B-cell non-hodgkins lymphoma

344
Q

omalizumab

A

IgE –> additional line of tx for severe asthma

FA (13 decks)

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