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Year 5 > Immuno Path flashcards > Flashcards

Immuno Path flashcards

1
Q

Different types of SCID

A

X-linked (unresponsive to cytokines - IL-2);
Low T, Low NK, High (non-functioning) B cell, Low Ig

ADA (presents 3 months);
Low T, Low B, Low NK, normal neutrophil

Reticular Dysgenesis (AK2);
Low ALL - presents very early
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2
Q

What is Bare Lymphocyte Syndrome 2

A

No CD4 cells –> cant class switch IgM to IgG/IgA

Normal CD8, absent/low CD4, HIGH IgM, Low IgG/IgA

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3
Q

CD40 mutation

A 
DEFECTIVE CD40 on T cell = no class switching
Normal CD9, Normal CD4, High IgM, Low IgG/IgA
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4
Q

Bruton

A

X linked, Tyrosine Kinase Gene
Stops Pre B cells maturing to B cells
Therefore No B cell or Igs, normal everything else

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5
Q

What ligand is commonly affected in LAD

A

CD18

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6
Q

Pathophysiology of CGD and treatment?

A

No oxidative killing, therefore NON-caseating granulomas form

NEGATIVE dihydrorhadanine and NBT

antibiotics, antifungals and interferon Gamma

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7
Q

What are primary innate immunodeficiencies susceptible to?

A

Staph Aureus
Enteric organisms
Candida Albicans
Aspergillus Flavus

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8
Q

What does NK cell deficiency predispose to

A

Viral infections and tumours

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9
Q

Primary innate immunodeficiency, recurrent atypical mycoplasma/ TB infections

A

IFN gamma receptor deficiency

Signalling failure; IL-12 and IFN gamma
(produced by macrophage and T cell) respectively
(receptor for T cell and macrophage) respectively

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10
Q

Inheritance for Kostmans vs Cyclical

A

AR vs AD

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11
Q

What is the most common complement immunodeficiency

A

C2, associated with SLE

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12
Q

Why are complement deficiencies associated with SLE

A

less removal of cellular debris gives NUCLEAR remnants causing ANA production –> SLE

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13
Q

Which organisms are complement deficiencies susceptible to>

A

NHS (encapsulated)

complement forms the attack complex which normally poked holes in the encapsulated organisms

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14
Q

CVID

A 
Common variable immunodeficiency
Presents later in life with IgG/IgA/IgE deficiency alongside;
another AI disease
Lung, liver, kidney problems
 \+ LYMPHOMA
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15
Q

What are the co-factors for HIV binding to T cells

A

CD20 (main), CCR5, CXCR4

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16
Q

What ligands does HIV use to bind?

A

gp120, gp41

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17
Q

What are the screening tests if you suspect someone has HIV

A

1) ELISA

2) rapid screening test (less sensitive)

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18
Q

What is diagnostic for HIV?

A

Western Blot (for HIV antibodies)

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19
Q

What are the most important HLAs for transplant matching

A

DR>B>A

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20
Q

How many HLA mismatches are too many?

A

> 6

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21
Q

what is the chance a sibling will have x amount of HLA matches

A 
25% = 6 MM
50% = 3 MM
25% = 0 MM
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22
Q

What are the types of graft rejection?

A

Hyperacute - preformed antibodies to ABO
Acute 1) T cell mediated; interstitial inflammation
2) Antibody mediated - vasculitis
Chronic - can be immune or non immune

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23
Q

Which drugs are used for prophylaxis of transplant rejection

A

1) antiproliferative - azathioprine (pro-drug which turns into 6-mercaptopurine) OR mycophenalate mofetil
2) Cell signalling inhibitor (calcineurin) such as tacrolimus or cyclosporin)
3) Steroids

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24
Q

C3 deficiency + lipodystrophy + glomerulonephritis?

A

C3 nephritic factor (antibody to C3 convertase)

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25
Q

Polygenic autoinflammatory condition?

A

Familial mediterranean fever (FMF)

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26
Q

What is FMF (symptoms, pathophysiology) + treatment

A

mutation in the MEFV gene causing failure to regulate cryopyrin regulation of neutrophils —»> which means the inflammasome complex doesn’t work

fever + serositis (lung, mesenchyma) every 46-96 hours
+ AA deposits long term

Treatment; colchicine (neutrophil inhibitor) + anti IL-1 and anti TNF alpha (anakinra and etanercept)

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27
Q

Name 3 monogenic autoimmune disease

A

APS-1/APECED - autoimmune polyendocrinopathy, candidiasis, ectodermal dystrophy
IPEX - Immunodysregulation polyendocrinopathy enteropathy X-linked
ALPS - autoimmune lymphoproliferative disorder

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28
Q

APS-1

A

Defect in the AIRE protein (means thymic central tolerance is defective so autoreactive T cells fly around causing loads of random AI disease)

Autosomal recessive

Hypoparathyroid + Addisons + candida infections (as also against IL17)

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29
Q

What do treg cells have on their surface

A

CD3 (all T cells), Foxp3 and CD25

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30
Q

What is IPEX

A

IPEX = immune dysregulationpolyendocrinopathy enteropathy.
- treg malfunction because Foxp3 is mutated = lack of PERIPHERAL tolerance

3 Ds; polyendocrine and enteropathy;
Dermatitis, Diabetes and Diarrhoea

X-linked

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31
Q

ALPS

A

Autoimmune lymphoproliferative syndrome
- failure to apoptose lymphocytes vis FAS pathway
- CD4 negative and CD8 negative BOTH present
- High lymphocyte count
cytopaenia e.g. haemolytic anaemia and LYMPHOMA

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32
Q

Crohns disease gene

A

IBD1 - NOD2

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33
Q

HLA associations; seronegative arthropathies

A

PEAR; B27

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34
Q

HLA associations; goodpastures

A

DR2/DR15

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35
Q

HLA associations; Graves

A

DR 3

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36
Q

HLA associations; SLE

A

(DR )2,3, SLE

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37
Q

HLA associations; T1DM

A

DR3/4

38
Q

HLA associations; Rheumatoid arthritis

A

DR4

39
Q

Hypersensitivity disorders: atopic dermatitis

A

type 1

40
Q

Investigations for allergy

A

acute; mast cell tryptase

otherwise

1) RAST; can rule out allergy
2) Skin prick
3) Gold standard challenge test (in hospital)

41
Q

Hypersensitivity disorders: serum sickness

A

type 3, reaction to proteins (penicillin) in antiserum

42
Q

Hypersensitivity disorders: polyarteritis nodosa

A

type 3

43
Q

Hypersensitivity disorders: SLE

A

type 3

44
Q

Hypersensitivity disorders: T1DM

A

type 4

45
Q

Hypersensitivity disorders: MS

A

type 4

46
Q

Hypersensitivity disorders: Contact dermatitis

A

type 4

47
Q

Hypersensitivity disorders: mantoux test

A

type 4

48
Q

Autoantibodies: Antiphospholipid syndrome

A

cardiolipin, lupus anticoagulant, B2 glycopeptide

49
Q

Autoantibodies: AI hepatitis

A

anti LKM-1 and anti smooth-muscle

50
Q

Autoantibodies: congenital heart block in children when mothers have SLE

A

anti-Ro

51
Q

Autoantibodies: Dermatitis herpertiformis

A

anti-endomysial

52
Q

Autoantibodies: Diffuse cutaneous scleroderma

A

anti-topoisomerase (SCL-70) - nucleolar pattern

53
Q

Autoantibodies: Sjogrens

A

anti ENAs; anti-Ro, anti-La (speckled pattern)

54
Q

Autoantibodies: Myasthenia gravis

A

anti-Ach

55
Q

Name live attenuated vaccines

A

MMR VBOY
Measles
Mumps
Rubella

VZV (no HIV)
BCG
Other; Sabin (polio) and typhoid
Yellow fever (no HIV)

56
Q

Name conjugate vaccines

A

NHS

pneumococcal is polysaccharide

57
Q

Name toxoid vaccines

A

Diptheria, Tetanus

58
Q

Name component vaccines:

A

HPV (capsid), HepB (HbS antigen), Influenza (HG/NA)

59
Q

Name DNA/ RNA vaccines

A

COVID - target spike protein

60
Q

Name vaccine adjuvants

A

aluminium salt (ALUM) - B cell maturation
CpG
IL2 - for Hep B (t cell)

61
Q

T cell replacement therapies (3)

A

1) viral (leukaphoresis, sensitise, expand, return)
2) tumour (biopsy, isolate, expand, return)
3) CAR-T (leukaphoresis, prime against CD19, return to kill B cells - ALL and NHL)

62
Q

General IVIG replacementt

A

1) primary immunodeficiencies

2) secondary immunodeficiencies (CLL, MM, BM transplant)

63
Q

Interferon therapies

A

IFN alpha; hep B and C + CML
IFN Beta; Behcets
IFN gamma: CGD

64
Q

Melanoma treatments

A

checkpoint blockers;

  • anti PDL1 = PEMBROLIZUMAB/ nivolumab
  • anti CTLA4 = IPILIMUMAB
65
Q

name anti proliferative agents (4)

A

azathioprine, mycofenalate mofetil, cyclophosphamide, methotrexate

66
Q

MOA and considerations: azathioprine

A

used for transplant prophylaxis
prodrug –> 6 mercaptopurine (BM suppression)
T>B cells
antimetabolite (purine)
need to measure TPMT levels – allopurinol

67
Q

MOA and considerations: mycofenalate mofetil

A

used for transplant prophylaxis
T>B cells
antimetabolite (guanine)
complication; progressive multifocal leukoencephalopathy (reactivating the JC virus)

68
Q

MOA and considerations: cyclophosphamide

A

B > T cells
connective tissue, vasculitis, cancer
Haemorrhagic cystitis

69
Q

MOA and considerations: Methotrexate

A

folate
Rheumatoid arthritis, psoriasis, crohns + abortions
BM suppression

70
Q

Name cell signalling inhibitors:

A

Tacrolimus, Cyclosporin, Sirolimus (MTOR), Tofacetinib (JAK-STAT), Apremilst

71
Q

MOA and considerations: Tacrolimus/ Cyclosporin

A

Rejection prophylaxis in transplants
calcineurin inhibitor
nephrotoxic and ototoxic
cyclosporin –> gingival gum hypertrophy

72
Q

Name agents directed at cell surface antigens

BAM No Receptor Today!

A

Basilixumab (anti CD25) - allograft rejection
Anti-thymocyte (allograft rejection)
Muromonab (mouse, anti-CD3) - active allograft rejection

Natalizumab (for relapsing remitting MS)

Tocilizumab (anti IL6) - RA, doesnt work in PA
Abatacept (anti-CTLA4) - RA and melanoma

Rituximab (anti CD20) - depletes mature B cells for LYMPHOMA

73
Q

Name agents against cytokines

A

anti TNF alphas (infliximab, adalimumab, Certolizumab, Golimumab) - RA + PEAR

Etanercept (TNFa and TNFb) - RA + PEAR
Secukinumab (anti IL17) - 3rd line Ank,Spon + psoriasis
Ustekinumab (anti-IL12 and IL23) - psoriasis

Denosumab (anti-RANKL) - osteoporosis

74
Q

Autoantibodies: dermato/polymyositis

A

Anti-Jo-1 (tRNA synthetase)

75
Q

HLA association: Coeliac

A

DQ 2 and 8

‘I ate too much at Dairy Queen’

76
Q

What can be used as a marker of SLE severity?

A

C3, C4 (C4 depletes first, if severe C3 depletes)

77
Q

Treatment for Ankylosing Spondylitis

A

NSAIDs –> Etanercept (anti TNFa/b) –> Secukinumab (anti IL17)

78
Q

What causes a post transplant lymphoproliferative disease?

A

EBV

79
Q

What does rituximab do?

A

Cell surface antigen blocking of CD20
Used in LYMPHOMA ( and ALL)
Rheumatoid arthritis and SLE

80
Q

What is the Gag protein (HIV)

A

intrastructural support

81
Q

C1 inhibitor deficiency

A

hereditary angioedema

82
Q

Which drug commonly can cause anaphylaxis (drug reaction)

A

ACEi (think hypertension)

83
Q

Mutation for HIV resistance

A

CCR5-delta 32

84
Q

Which immune cell produced in the bone marrow, migrates to perform oxidate and non oxidative killing methods?

A

neutrophil

85
Q

A teenage girl who gets an erythematous rash when cross-country running

A

Cold urticaria

86
Q

A 60 year old lady with recurrent chest infections, reduced total serum protein and ITP. What is the diagnosis

A

CVID

87
Q

What immunodeficiency is susceptible to mycobacteria?

A

IFN gamma deficiency

88
Q

What does common gamma chain mean and thus what is the related immunodeficiency

A

IL2 and X-linked SCID

89
Q

What cell type do antibodies bind to in antibody mediated solid organ transplant rejection?

A

Endothelial

the other is T cell mediated

90
Q

ALUM effect of vaccine

A

helps B cell maturation

91
Q

CpG effect on vaccine

A

Activates Toll-like receptors on APCs to increase costimulation

92
Q

What adjuvant can you give to people receiving the HepB vaccine

A

IL 2

Year 5 (7 decks)

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