Immune basics Flashcards

1
Q

adaptive immune cells

A

CD4 T cells
B cell
CD8 T cell

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2
Q

Innate immunity

A

neutrophil
macrophage
natural killer cells

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3
Q

how does measles affect immune response ?

A

modify
decrease production and function of t cells

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4
Q

secondary immunodeficiencies

infection
biochemical
malignancy
drugs

A

HIV
measles
mycobacterial

malnutrition
diabetes
Renal insufficiency

myeloma
leukaemia
lymphoma

steroids
antiproliferative
cytotoxic agents

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5
Q

immunodeficiency - when can it be physiological?

A

neonates
elderly
pregnancy

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6
Q

what can be a cause of secondary immunodeficiency

A
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7
Q

3 condition that cause defective neutrophil production - do not produce

A

reticular dysgenesis
kostman
cyclic neutropenia

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8
Q

reticular dysgenesis

A

reticular dysgenesis - severe scid - autosomal recessive nothing is produced

no myeloid / lymphoid

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9
Q

kostmann

A

autosomal recessive
congenital neutropenia

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10
Q

cyclic neutropenia

A

ELA-2 neutrophil elastase deficient

autosomal dominant

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11
Q

leucocyte adhesion deficiency

A

the Cd18 b2 integrin is deficient

can’t bind to ICAM-1 so cannot exit blood

very high neutrophil count
nitrofurantoin test positive
no pus

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12
Q

hwo do neutrophils normally enter tissue?

A

b2 integrin - CD18 combines with cd11a = LFA-1
lymphocyte function associated antigen 1

LFA-1 binds to ICAM-1

= neutrophil transmigration and adhesion

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13
Q

b2 integrin ______, binds to ____ which forms ______

this then binds to intracellular adhesion molecule 1 on endothelial cells to mediate what?

A

CD18 combines with CD11a to produce LFA-1

LFA-1 binds to ICAM-1

allowing for neutrophil transmigartion and adhesion

in LAD this is stopped due to deficient b2 integrin = high neutrophilia, no pus formation

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14
Q

4 mechanisms of phagocyte deficiency?

A

1) failure to produce
2) failure to leave blood (transmigration)2
3) failure to produce cytokines
4) failure to perform oxidative killing

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15
Q

failure of oxidative killing?

A

chronic granulomatous disease

NADPH impacted (deficient)
= no ROS (HOCL)

persistent neutrophil accumulation = inflammation= pus = granuloma

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16
Q

what kind of bacteria are you most susceptible to in CGD?

A

PLACESS

catalase positive

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17
Q

catalase positive bacteria?

A

Pseudomonas,
Listeria,
Aspergillus,
Candida,
E.Coli,
Staph Aureus,
Serratia

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18
Q

cytokine cycle macrophages and t cells?

A

IL12 produced
>
T cell stimulated
>
IFN gamma
>
back on macrophage
>
TNF-a / free radical

= NADPH oxidase = ROS

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19
Q

which cytokine do macrophages rpoduce that stimulates t cells?

A

IL12

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20
Q

t cell stimulates what?

A

ifn-y which then stimulates macrophages

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21
Q

do macrophages or t cells produce TNF?

A

macrophages, which get there signalling from IFN-y

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22
Q

what organisms infect macrophage?

A

atypical mycobacteria

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23
Q

nitroblue tetrazolium blue test

A

is a test for oxidation - hydrogen peroxide

so no colour change in CGD - usually would be yellow > blue

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24
Q

main test for CGD?

A

dihydrorhodamine

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25
Q

phagocyte deficiency what type of infection?

A

skin and mouth

mycobacterial infection - TB, atypical mycobacteria

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26
Q

neutrophil production

A

reticular dysgenesis
kostmann
cyclic neutropenia

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27
Q

For each of the following diseasesm state the expected neutrophil count, leucocyte adhesion markers, NBT/DHR test and presence of pus:

kostmann

A

neutropenia / no neutrophils
normal 3
no colour change
no pus

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28
Q

For each of the following diseases state the expected neutrophil count,
leucocyte adhesion markers,
NBT/DHR test
presence of pus:

LAD

A

neutrophilia - very high
absent cd18
colour change blue>yellow
absent

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29
Q

For each of the following diseases state the expected neutrophil count,
leucocyte adhesion markers,
NBT/DHR test
presence of pus:

CGD

A

normal
present
no colour change
present pus

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30
Q

For each of the following diseases state the expected neutrophil count,
leucocyte adhesion markers,
NBT/DHR test
presence of pus:

IL12/IFn-y

A

neutrophil count normal
adhesion markers present
normal
present

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31
Q

phagocyte deficiencies - management?

A

IFN-y if CGD
IgG
SCT

aggressive management of infection

32
Q

what is main risk with NK deficiency?

A

increase risk of viral infection
HSV,CMV,EBV, VZV

33
Q

if nk deficient?

A

antiviral - aciclovir
cytokine - IFN-a

HSCT

34
Q

recurrent infection with no neutrophils on FBC?

A

kostmann

35
Q

high neutrophil, no pus formation
recurrent infection?

A

LAD

36
Q

recurrent infection, hepatsplenomegaly and abnormal DHR

A

cgd

37
Q

infection w atypical mycobacteria?
normal fbc?

A

ifn-y

38
Q

regulatory protein in alt pathway?

A

factor h

39
Q

main consequence of complement deficiency?

A

encapsulated bacter infection risk

NHS

neisseria meningitides
haemophilus influenzae
streptococcus pneumoniae

40
Q

SLE and complement deficiencies?

A

c2

classical pathway promotes phagocyte mediated killing of apoptotic cells

ineffective clearance = increase in self antigen and antibody

41
Q

why is classical pathway associated w SLE?

A

immune complex clearance
clearance of apoptotic cells : when this doesn’t happen there is an increase in self antigen

42
Q

clinical phenotype of complement deficiency?

A

SLE/C2
skin disease

43
Q

what does functional complement deficiency mean?

A

active lupus = production of immune complexes
= consumption of complement
= functional deficiencyof c3, c4 as they are being used up

44
Q

nephritic factors?

A

autoantibodies directed against

c3 convertases
= c3 activation and consumption

45
Q

how to test classical pathway?

A

ch50
assay - c1,2,3,4,5-9

46
Q

how to test altrnative pathway?

A

b,d,properidin

c3, c5-9

47
Q

severe childhood onset sle w normal c3, c4

A

c1q

48
Q

causes of secondary immunodeficiency
drugs

A

corticosteroids
cytotoxic

49
Q

causes of secondary immunodeficiency

A

infectionn
biochemical - malnutrition, zinc/iron, renal impairment

malignancy
drugs

50
Q

what disease is usually associated with nephritic factors?

A

glomerulonephritis: membranoproliferative

51
Q

SCID - what is the gene in reticular dysgenesis

A

adenylate kinase 2
mitochondrial energy metabolism enzyme

52
Q

most common type of SCID?
what mutation
causes?

A

x-linked

xq13.1

Il-2R deficient
cannot respond to cytokines

53
Q

x linked SCID cell counts

A
54
Q

clinical phenotype fo SCID

A

unwell by 3 months - maternal IgG dissapates
fail to thrive
diarrhoea
skin disease

55
Q

function of cd4+ cell?

A

full b cell response
cd8 t cell monitor

56
Q

what is diGeorge

A

22q11.2
failure of development of pharyngeal pouch
t cell vibes

57
Q

DiGeorge Catch-22

A

cardiac abnormality - tet of fallot
abnormal face
thymic aplasia
cleft
hypocalcaemia/ hypopara
22 chromosome

58
Q

CD8 cytotoxic killing

A

fas ligand
granzymes, perforins

59
Q

underdeveloped thymus ?

A

b cell is fine
t cell is low

60
Q

MHC class 2

A

Bare lymphocyte syndrome
Cd4+ cannot be selected
= cd4+ deficiency

61
Q

bare lymphocyte syndrome type 2
cell counts
Cd8
Cd4
B
IgG

A

normal
low
normal
low (plasma need t cell interaction cd4)

62
Q

ADA
cell counts
Cd8
Cd4
B
IgG

A

low T
low B
low NK

63
Q

x linked scid
cell counts
T
B
NK

A
64
Q

t lymphocyte deficiency ?

A

viral infections
fungal
bacterial
malignancy

65
Q

how to investigate t cell deficiency?

A

WCC
lymphocyte subset
Immunoglobulins
functional test of t cell
HIV

66
Q

Describe the typical levels of CD4, CD8, B cells, IgM and IgG that you would expect to see in the following diseases:

SCID

A

CD4- low
CD8-low
B-can be normal / low
IgM low/normal
IgG low

67
Q

Describe the typical levels of CD4, CD8, B cells, IgM and IgG that you would expect to see in the following diseases:
Di George

A

low
low
normal
IgM normal
IgG low/normal

68
Q

BLS type 2
Describe the typical levels of CD4, CD8, B cells, IgM and IgG that you would expect to see in the following diseases:

A

low,
normal
normal
IgM normal
IgG low

69
Q

Fc constant region determines class of immunoglobulin t/f?

A

true this is heavy chain

70
Q

effector function of immunoglobulin?

A

constant region of hevay chain
Fc

71
Q

role of antibody?

A

identify
interact w complement, phagocyte, NK cell

72
Q

Bruton x link hypogammaglobulinaemia

A

prevent maturation of b cell

b cell tyrosine kinase gene
BTK

absence of mature b cells - absence of antibodies

73
Q

boy 4 has had 5 otitis media episodes this year, he was recently discharged from hospital after admission with severe pneumonia

presenting to GP with fungal growth on toe
in 5th centile of growth

A

x linked hypogammaglobulinaemia

74
Q

clinical features of antibody deficiency

A

bacterial infections
toxins
viral infections

75
Q

b cell deficiency

A

wcc
lymphocyte subset
serum Immunoglobulin

functional b cell function test
igG antibody against s.pneumoniae

igG is surrogate marker for cd4+ t helper cell fynction

76
Q

2

A