Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Path > Immuno > Flashcards

Immuno Flashcards

1
Q

What are most deaths within 5 days of burns injuries related to?

A

Infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Reticular dysgenesis mutation and side effect

A

Adenylate kinase 2 gene (energy metabolism of mitochondria)

Bilateral sensorineural deafness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Kostmann syndrome problem and mutation

A

Failure of neutrophil maturation in children

HCLS1-associated protein X-1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Condition in which there is episodic neutropaenia every 4-6 weeks, and parents will have condition (autosomal dominant). Genetics and treatment.

A

Cyclic neutropaenia

Mutation in neutrophil elastase ELA-2

Responds to G-CSF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Outline leukocyte adhesion deficiency cause

A

CD18 deficiency

Usually expressed on neutrophils to help bind to ICAM-1 on endothelial cells

Lack this, so fails to exit bloodstream

  • High neutrophil count in blood
  • Absence of pus formation
  • Delayed umbilical cord separation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Outline chronic granulomatous disease cause and features

A

NADPH oxidase deficiency - oxygen conversion to superoxide needed for hypochlorous acid

Impaired killing of intracellular microorganisms

Granulomas, lymphadenopathy, hepatosplenomegaly
Susceptible to catalase positive bacterie i.e. PLACESS (Pseudomonas, Listeria, Aspergillus, Candida, E. coli, S. aureus, Serratia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Chronic granulomatous disease investigations and definitive treatment

A

Negative nitro-blue tetrazolium test (NBT) normally turns from yellow to blue following interaction with hydrogen peroxide
In CGD it stays yellow

Dihydrorhodamine (DHR) flow cytometry test (gold standard). Oxidised by hydrogen peroxide to rhodamine, which is fluorescent, if normal

IFN-gamma therapy
HSCT in general for phagocyte deficiencies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What problems can arise if there is a deficiency of IL-12 and IFN-gamma, and their receptors and why?

A

Susceptible to mycobacteria, BCG and salmonella

Normally:
• Infected macrophages produce IL-12 which leads to stimulatesd production of TNF-alpha and free radicals
• Activates NADPH oxidase

This oxidative pathway fails if you have deficiencies earlier on. Unable to form granulomas.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What type of microorganisms are recurrent in Factor B/I/P deficiency (alternative pathway)?

A

Encapsulated bacteria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What problems can arise with deficiency in the early classical pathway (C1/2/4), and which is the most common?

A
  • Immune complexes fail to activate complement
  • Failed clearance of apoptotic cells
  • Increase in self-antigens -> autoantibodies
  • Deposition of immune complexes

C2 most common deficiency - almost all have SLE, but C3 and C4 (goes down first) used to monitor SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is C1 esterase deficiency also known as?

A

Hereditary angioedema

C4 may also be low

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What causes secondary antibody dependent deficiency in the classical pathway?

A

Active lupus

Persistent production of immune complexes and consequent depletion of complement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Which complement does MBL deficiency involve and how does it affect people?

A

C2 and C4

Increased infections in immunocompromised

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What non-pathogenic disease are patients with C3 deficiency at an increased risk of developing?

A

Connective tissue disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Which factors stabilised C3 convertases leading to glomerulonephritis and lipodystrophy

A

Nephritic factors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What problems arise if there is a defect in the terminal common pathway

A

Failure to produce the membrane attack complex (MAC)

Can’t use complement to lyse encapsulated bacteria

Susceptible to N. meningitis, S. pneumonia, H. influenzae (vaccinate against these)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Most common type of SCID

A

X-linked

Mutation of IL2 receptor gamma chain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

When does SCID present and why

A

Presents 3-6 months after birth, protected by IgG from mother across placenta, then colostrum, before

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

SCID investigation findings

A

Flow cytometry - low B/T/NK cells

CXR - absent thymic shadow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Second most common type of SCID and its treatment

A

Adenosine deaminase deficiency

PEG-ADA enzyme replacement therapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Cardiac abnormalities (tetralogy of fallot)
Abnormal facies (high forehead, low-set ears)
Thymic aplasia
Cleft palate
Hypoparathyroidism

condition, genetics, other features

A

DiGeorge syndrome

CATCH 22
22q11.2 deletion

(also developmental delay, PCP infection, atypical viral infection)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

DiGeorge syndrome investigation findings and management

A

Low T cells, normal B cells (immune function improves with age)

Thymic transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Bare lymphocyte syndrome type II

  • what is absent/low
  • condition it is associated with
A

Regulatory protein issue

Absent MHC class II
CD4+ deficiency
No IgG or IgA (no class switching)

Association with sclerosing cholangitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Wiskott-Aldrich Syndrome mutation effect, features, Ig levels

A

Mutation affecting actin cytoskeleton arrangement, needed for T cell-APC interaction

Eczema, risk of lymphoma

High IgA, IgE
Low IgM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Outline Bruton’s X-linked agammaglobulinaemia (XLA) cause and features

A

Defective B cell tyrosine kinase gene
Pro-B cell doesn’t mature into pre-B cell

All immunoglobulins are low/gone (none after around 3 months), B cells are low

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Outline selective IgA deficiency features

A

Most common primary antibody deficiency
• 2/3 asymptomatic
• Recurrent sinopulmonary infections, GI infections, risk of GI cancers
• Anaphylaxis with exposure to blood products (with IgA)

Low IgA (sometimes normal, as mainly low on mucosal surfaces)
Sometimes high IgE (compensate for low IgA, leading to allergic reactions)
Normal B cell levels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Selective IgA deficiency management

A

Depends on manifestation e.g. ABx for infections

Vaccination prophylaxis (only deficiency where this works)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Mutation in CD40L in activated T cells condition

A

Hyper IgM syndrome

CD40 involved in T-B cell communication
Inability of B cells to class switch
Production of only IgM

X-linked

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Outline common variable immune deficiency, Ig levels and features

A

Group of disorders with unknown disease mechanism

Low everything apart from B cells and IgM

  • Bronchiectasis
  • Autoimmune disease
  • Granulomatous disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What does MEFV encode?
What does it interact with?
What diseases are mutations linked to?

A 
Encodes pyrin (marenostrin)
Interacts with ASC -> procaspase 1 in the inflammasome complex
Mutations linked to autoinflammatory diseases e.g. Mediterranean fever
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What does the NALP3/cryopyrin-inflammasome complex bind to in neutrophils?

A

ASC, activating procaspase 1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What type of pathogens are people with complement deficiencies susceptible to?

A

Encapsulated bacterial infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What tests are used to diagnose complement deficiencies?

A

CH50 and AP50

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q 
CH50, AP50, C3, and C4 levels in the deficiencies of the following:
• C1q
• Factor B
• C9
• SLE
A

C1q - low CH50
Factor B - low AP50
C9 - both low
SLE - both normal (CH50 may be low)

C3 and C4 only low in SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What types of pathogens are people with T cell deficiencies more susceptible compared to antibody/CD4 T cell deficiencies?

A

Viral and fungal, as opposed to bacterial

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Complement deficiencies general management

A

Vaccinate
Prophylactic Abx
Screen family

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What is a type I hypersensitivity disorder and what is an important symptom?

A

Reaction to allergen re-exposure

IgE cross-links mast cells - histamine release

Anaphylaxis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q 
What group are these diseases in?
Atopic dermatitis (infantile eczema)
Food allergy
Oral allergy syndrome
Latex food syndrome
Allergic rhinitis
Acute urticaria
A

Type I hypersensitivity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Atopic dermatitis defect. What does it also predispose to?

A

Defects in beta defensin

Predisposes to S. aureus superinfection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What causes oral allergy syndrome and how is it treated

A

Exposure to allergen e.g. birch pollen, seeded fruit

Induces allergy to food

If ingested, wash mouth and take antihistamines

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What food causes latex food syndrome?

A
  • Chestnut
  • Avocado
  • Banana
  • Potato
  • Kiwi
  • Papaya
  • Eggplant
  • Mango
  • Wheat
  • Melon
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q 
Are the following IgE or cell-mediated:
• Anaphylaxis
• Oral allergy syndrome
• Coeliac
• Atopic dermatitis
A

Anaphylaxis - IgE
OAS - IgE
Coeliac - cell-mediated
Atopic dermatitis - both

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What can trigger non-IgE mediated mass cell degranulation, leading to anaphylaxis?

A

NSAIDs
IV contrast
Opioids
Exercise

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

4 differentials of anaphylaxis

A

Hereditary angioedema (C1 inhibitor deficiency)
ACEi induced angioedema (lips and tongue)
Acute anxiety
Urticaria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

What happens when a1, b1, and b2 receptors are stimulated?

A

a1 - vasoconstriction
b1 - increased HR and contractility
b2 - bronchodilation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Anaphylaxis treatment algorithm in adults

A
  1. Lie flat (with or without legs elevated)
  2. IM adrenaline 1mg/mL 1:1000
  3. High flow 100% oxygen
  4. IV fluid challenge (crystalloid)
  5. IV chloramphenamine 10mg
  6. IV hydrocortisone 200mg (prevents rebound anaphylaxis)

(call ambulance after using epipen)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

What does a skin prick test show and how?

A

Wheal >=2mm

IgE-mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What should you do in preparation for a skin prick test?

A

Stop antihistamines 48 hours before (corticosteroids are ok to continue)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What does a RAST (quantative specific IgE to putative allergen) test measure, how does it compare to skin prick, and when is it indicated?

A

Measures levels of IgE against particular allergen in serum
Monitors response to treatment
Less sensitive/specific than skin prick

Use if can’t stop antihistamines, anaphylaxis hx, extensive eczema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

What do component-resolved diagnostics measure?

A

IgE response to a spefic allergen protein (rather than range of proteins from allergen)
e.g. in peanut, ara h 2 (anaphylaxis to peanut), ara h 8 (localised oral reaction to peanut)

Can confirm primary sensitisation and cross reaction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

What is the gold standard test for food allergy

A

Challenge test

Double-blind oral food challenge

Increasing volumes of offending food are ingested under close supervision

Risk of severe reaction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

When should tryptase be measured following anaphylaxis?

A

ASAP
Ideally within 1-2 hours, no later than 4 hours
Specialist follow-up for baseline may be required

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

What is a type II hypersensitivity disorder?

A

IgG or IgM reaction against cells or extracellular matrix

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Which antigens are targeted in the following type II hypersensitivity disorders:
• Haemolytic disease of the newborn
• Autoimmune thrombocytopaenic purpura
• Goodpasture’s syndrome
• Pemphigus vulgaris
• Myasthenia gravis
• Acute rheumatic fever
• Eosinophilic granulomatosis with polyangitis (Churg-Strauss)
• Granulomatosis with polyangitis (Wegener’s)
• Microscopic polyangitis

A
  • HDN - erythrocytes
  • ATP - Glycoprotein IIb/IIIa on platelets
  • Goodpasture’s - collagen type IV (BM, non-collagenous domain)
  • PV - epidermal cadherin
  • MG - ACh receptor
  • Acute rheumatic fever - M proteins of Group A Strep
  • eGPA - myeloperoxidase
  • GPA - proteinase 3
  • MPA - myeloperoxidase
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Pathology, diagnosis, and treatment of haemolytic disease of the newborn

A

Maternal IgG attacks neonatal erythrocytes - reticulocytosis and anaemia

Positive direct Coombs test

Maternal plasma exchange, exchange transfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Diagnosis and treatment of autoimmune haemolytic anaemia

A

Positive direct Coombs test, anti erythrocyte cell ab

Steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Goodpasture’s syndrome pathology and diagnosis

A

Glomerulonephritis, pulmonary haemorrhage

Anti glomerular basement membrane antibody
Linear smooth fluorescent staining of IgG on BM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Non-tense skin blisters, bullae
Fluorescent IgG deposition
Condition?

A

Pemphigus vulgaris

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Graves treatment

A

Carbimazole and propylthiouracil

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

Myasthenia gravis diagnosis and treatment

A

Anti-ACh-R ab, abnormal ECG
Tensilon test - ACh esterase inhibitor injection, improvement in symptoms

Neostigmine, pyridostigmine (IVIG if serious)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

What is used for diagnosis of acute rheumatic fever?

A

Jones criteria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

What can pernicious anaemia lead to if left untreated?

A

B12 deficiency

Optic neuropathy
Peripheral neuropathy
Subacute combined degeneration of the spinal cord (sensory deficit, weakness, ataxia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

p-ANCA against myeloperoxidase, asthma, heart and kidney disease

A

eGPA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

c-ANCA against proteinase 3, lung cavitations and haemorrhage, crescentic glomerulonephritis

A

GPA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

p-ANCA against myeloperoxidase, purpura, livedo reticularis

A

Microscopic polyangitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

What are 3 types of antinuclear antibodies?

A

anti-

dsDNA
ENA (extractable nuclear antigen)
cytoplasmic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

What does the presence of anti-dsDNA suggest?

A

SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

Autoimmune polyendocrinopathy candidiasis-ectodermal dystrophy is caused by a mutation in which gene?

A

AIRE (autoimmune regulator)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

Immunodysregulation, polyendocrinopathy, enteropathy, X-linked syndrome is caused by a mutation in which gene?

A

FOXP3 - defective Treg cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

What are type III hypersensitivity disorders?

A

IgG or IgM immune complex mediated tissue damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

IgM against IgG +/- hep C antigens, joint pain

Condition?

A

Mixed essential cryoglobulinaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Serum sickness cause and diagnosis

A

Reaction to proteins in antiserum (blood serum with antibodies) from non-human source or medications (most commonly penicillin)

Decreased C3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

High WCC, ESR and CRP
‘Rosary sign’ small aneurysms on angiography
Systemic ischaemia, livedo reticularis, nodules
Hep B and C association

Condition?

A

Polyarteritis nodosa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

SLE basic pathophysiology

A

Failure of immune complex and apoptotic cell clearance

Shift from Th1 to Th2 -> B cell hyperactivity -> autantibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Which autoantibodies are involved in SLE (6)?

A 
Anti-Ro
Anti-La
Anti-Sm
Anti-U1-RNP
Anti-dsDNA
Anti-histones
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

What can hydralizine, procainamide and isoniazid all cause?

A

Drug-induced SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

What is useful for SLE disease monitoring?

A

anti-dsDNA

C4 first to drop

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

In SLE, what is the appearance of the basement membrane antibody on immunofluorescent exmination of the skin?

A

Granular lumpy-bumpy appearance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

What is a type IV hypersensitivity disorder?

A

Delayed T-cell mediated hypersensitivity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

Name 5x type IV hypersensitivity disorders?

A 
T1DM
MS
Rheumatoid Arthritis (also type III - IgM vs Fc of IgG)
Contact dermatitis
Crohn's disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

What medical test can ellicit a type IV hypersensitivity reaction?

A

Mantoux test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

T1DM antigens targeted

A

Pancreatic beta cell proteins (glutamate decarboxylase)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

MS antigens targeted

A

Oligodendrocyte proteins (myelin basic protein, proteilopic protein)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

MS diagnosis

A

Oligoclonal bands of IgG from CSF on electrophoresis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

Rheumatoid arthritis HLA associations

A

HLA-DR1

HLA-DR4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

Which 2 enzymes are implicated in RA pathogenesis?

A

PAD2
PAD4

(Peptidylarginine deiminases)

They generate citrullinated autoantigens (arginine -> citrulline). Enzymes present in neutrophils and monocytes - so RA in inflamed synovium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

More likely to get RA, SLE, T1DM, decreased T cell activation.
Condition?

A

PTPN22 (Protein Tyrosine Phosphatase Non-Receptor Type 22)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

RA diagnosis

A

Anti-cyclic citrullinated protein - most specific (and generally most accurate)

Rheumatoid factor - slightly more sensitive but can be found in people with other AI diseases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q

Causes of contact dermatitis

A

Poison ivy
Nickel
Latex

90
Q

What is a major genetic risk factor for Crohn’s disease?

A

NOD2 cystosolic protein on chromosome 16

91
Q

Crohn’s diagnosis

A

Biopsy of lesion

92
Q

HLA associations of the following:
Graves disease
T1DM
Coeliac

A

GD - HLA-DR3
T1DM - HLA-DR3/4
Coeliac - HLA-DQ2,8 (ate 8 too 2 much at Dairy Queen)

93
Q

SLE HLA associations

A

HLA-DR2
HLA-DR3

2-3, S-L-E

94
Q

Diseases associated with HLA-DR2

A

Multiple hay pastures have dirt

MS
Hay fever
Goodpasture’s syndrome
SLE

95
Q

Diseases associated with HLA-B27

A

PAIR - seronegative arthropathies

Psioriatic arthritis
Ankylosing spondylitis
IBD-associated arthritis
Reactive arthritis

96
Q

CTLA4 is receptor for what

A

Receptor for CD80/86 from T cells

Inhibitory signal to control T cell activation

97
Q 
Calcinosis
Raynaud's
Oesophageal dysmotility
Sclerodactyly
Telangiectasia

condition and other features

A

Limited cutaneous scleroderma

CREST syndrome

Primary pulmonary hyperation
Skin involvement up to forearms + perioral

98
Q

CREST syndrome diagnosis

A

Anti-centromere antibodies

99
Q

Diffuse cutaneous scledorema presentation

A

CREST + GIT + interstitial pulmonary disease + renal cysts

Skin beyond forearms

100
Q

Anti-topoisomerase
Anti-Scl-70
Anti-fibrillarin
Anti-RNA polymerase I-III

Nucleolar staining by indirect immunofluorescence

Condition?

A

Diffuse cutaneous scledorema

101
Q

Xerostomia (dry mouth)
Keratoconjunctivitis sicca (dry eyes)
Dry nose
Dry skin

Condition

A

Sjorgen’s syndrome

102
Q

Sjorgen’s syndrome diagnosis

A

Anti-Ro
Anti-La

Schirmer test - check for dry eye

103
Q

IPEX presentation apart from
Nail dystrophy
Alopecia universalis
Bullous pemphigoid

A

Diarrhoea
Dermatitis
Diabetes mellitus

104
Q

IPEX cure

A

BM transplant

105
Q

Visible manifestation of coeliac disease

A

Dermatitis herpetiformis - extensor surface rash

106
Q

Coeliac investigations

A

IgA anti-edomysial antibody (postive when eating gluten
IgA anti-transglutaminase antibody (specific, and most sensitive)
IgG anti-gliadin antibody (most persistant)

> =4 duodenal biopsies (gold standard, need to know baseline too)
Villous atorphy and enteropathy
20 intraepithelial lymphocytes

107
Q

Hashimoto’s thyroiditis targeted antigen

A

Thyroglobulin

Thyroperoxidase

108
Q

Dermatomyositis and polymyositis autoantibodies

A

Anti-Jo-1 (against t-RNA synthetase)

109
Q

Primary biliary cirrhosis autoantibody

A

Anti-mitochondrial antibody

110
Q

Which autoantibody is a speckled fluorescent appearance seen with?

A

Anti-U1RNP

111
Q

Human normal immunglobulin (IgG antibody replacement)

From how many donors?
Which 3 infections are always screened ford?
How often is it given?

A

> 1000 donors
Screen for HIV, Hep B, Hep C
Every 3-4 weeks

112
Q

What is specific immunoglobulin injection given for?

A

Post-exposure prophylaxis, passive vaccination

Ig for rabies, VZ, Hep B, tetanus

113
Q

What can the following recombinant cytokines be used to boost the immune response to?

Interferon alpha
Interferon beta
Interferon gamma

A

alpha - Hep B, Hep C, Hairy cell leukaemia, chronic myelogenous leukaemia, malignant myeloma

beta - Bechets

gamma - CGD

114
Q

Ipilimumab function

A

Antibody against CTLA4 (downregulatory immune checkpoint)

Allows T cell activation

Used for advanced melanoma

115
Q

Pembrolizumab/nivolumab function

A

Antibody against PD-1 (promotes T cell apoptosis, prevents Treg apoptosis)

Allows T cell activation

Used for advanced melanoma

116
Q

What are CD19-targeted (B cell) CAR T cells [therapy] useful for?

A

ALL

AML too

117
Q

John Cunnigham virus (JCV) can reactivate during immunosuppressive therapy. Which cells does it attack?

A

Oligodendrocytes

118
Q

Prednisolone mechanism of action

A
  • Inhibits phospholipase A2
  • Reduced prostaglandin synthesis
  • Inhibits phagocyte trafficking
  • Lymphopaenia
  • Decreased Ab production
119
Q

• Alkylates guanine (DNA nucleotide)
• Damages DNA
• Affects B > T cells
Medication

A

Cyclophosphamide

120
Q

Haemorrhagic cystitis
Bladder cancer
Teratogenic
Sterility

SE of which drug

A

Cyclophosphamide

121
Q

Mycophenolate mofetil mechanism of action

A
  • Inhibits IMPDH
  • Blocks de novo nucleotide synthesis of guanine
  • Affects T > B cells
122
Q

Azathioprine mechanism of action

A
  • Converted to 6-MP and 6-thioguanine
  • Prevent de novo purine synthesis and also halt replication of DNA
  • Affects T cells > B cells
123
Q

Allograft regimen

A

Mycophenolate, prednisolone, tacrolimus

124
Q

Methotrexate mechanism of action

A

Inhibits dihydrofolate reductase

Decreases DNA synthesis

125
Q

What is plasmapharesis and what is it used for?

A
  • Device separates whole blood into components
  • Plasma treated to remove immunoglobulins or replaced with colloid fluid
  • Reinfused

Severe antibody-mediated (Type II) disease
Antibody-mediated rejection

126
Q

What needs to be coadministered with plasmapharesis and why?

A

Anti-proliferative agent e.g. cyclophosphamide

Rebound antibody production limiting efficacy and risking anaphylaxis

127
Q

Tacrolimus and cyclosporin mechanism of action

A
  • Inhibits calcineurin
  • Reduced IL-2 transcription
  • Reduced T cell proliferation
128
Q

Immunosuppresant with gingival hypertrophy side effect

A

Cyclosporin

129
Q

Sirolimus mechanism of action

A

mTOR (protein kinase) inhibitor

Blocks clonal proliferation of T cells

130
Q

Tacrolimus, cyclosporin and sirolimus indication

A

Rejection prophylaxis in transplantation (renal)

131
Q

Tofacitinib mechanism and indication

A

JAK inhibitor
Suppresses STAT-1 dependent genes

Rhematoid arthritis
Psoriatic arthritis

132
Q

Apremilast mechanism and indications

A

PDE4 inhibitor
Decreased cytokine production

Psoriasis
Psoriatic arthritis

133
Q

Basiliximab mechanism

A

Anti-CD25 (alpha chain of IL-2)

134
Q

Abatacept mechanism and indication

A

CTLA4 Ig, binds to CD80/86
Inhibits T cell activation

Rheumatoid arthritis

135
Q

Rituximab mechanism

A

Anti-CD20

Depletes mature B cells (not plasma cells)

136
Q

Natalizumab mechanism and indication

A

Anti-alpha4 integrin (binds to VCAM1) - mediates rolling of leukocytes

Relapsing-remitting MS

137
Q

Tocilizumab mechanism and indication

A

Anti-IL6

Castleman’s disease

138
Q

Muromonab mechanism and indication

A

Anti-CD3 on T cells (mouse monoclonal antibody (OKT3))

Active allograft transplant rejection

139
Q

Horse or rabbit-derived antibodies against human T cells and their precursors

A

Anti-thymocytes globulin (ATG)

140
Q

Daclizumab mechanism and indication

A

IL2 receptor antibody against CD25

Transplant rejection prophylaxis

141
Q

Efalizumab mechanism

A

Anti-CDIIa

142
Q

Alemtuzumab (Campath) mechanism

A

Antibody against CD52 on lymphocytes

143
Q

What does infliximab, adalimumab, certolizumab, and golimumab target and what are their indications?

A

Anti-TNF alpha

RA, AS, psoriasis, psoriatic arthritis, IBD

144
Q

How is etanercept different to other TNF alpha inhibitors?

A

It acts as a decoy receptor that binds to TNF anlpha

145
Q

Mechanism of ustekinumab and indications?

A

Anti-IL12 -> less Th1 diff -> less CD8+
Anti-IL23 -> less Th17 diff -> less B cells
Binds to p40 subunit

Psoriasis, psoriatic arthritis

146
Q

Secukinumab mechanism

A

Anti-IL17A

147
Q

Denosumab mechniasm, indications, and a side effect?

A

Anti-RANK ligand - inhibits osteoclast diferentiation

Osteoporosis, MM, bone mets

Avascular necrosis of jaw

148
Q

What are injection recall reactions?

A

Injection site reaction at previous sits

149
Q

Are injection site and infusion reactions IgE mediated?

A

Infusions are, injection generally more cellular

150
Q

How often is allergen desensitisation carried out and what is it useful for?

A

Escalate weekly until maximal dose
Maintenance dose monthly for 3-5 years

Bee venom, wasp venom, grass pollen, dust mite

Not food or latex

151
Q

Where are isografts, allografts, and xenografts from?

A

Isograft - twin
Allograft - same species
Xenograft - different species

152
Q

Which allografts can be taken from a living donor?

A

Bone marrow
Kidney
Liver

153
Q

How do T cells and B cells recognise antigens differently

A

T cells - antigen with MHCs on APCs

B cells - just antigen

154
Q

Importance of HLA type
A
B
DR

A

DR > B > A

155
Q

HLA chromosome

A

Chr6

156
Q

Where do T and B cells cause damage in a renal transplant?

A

T cells - interstitial

B cells - endothelial

157
Q

Difference between direct and indirect transplant recognition

A

Direct
• Donor APC presenting its own antigen/MHC to recipient T cells
• Mainly acute rejection

Indirect
• Recipient APC presenting donor antigen to recipient T cells
• The normal function of immune system
• Mainly chronic rejection

158
Q

Which 3 cells infiltrate the graft in to the effector phase of graft rejection?

A

CD4+

Then cytotoxic T cells punch holes in target cells

Then macrophages get involved

159
Q

Hyperacute transplant rejection time and mechanism

A

Minutes - hours

Preformed antibodies activate complement

160
Q

Acute transplant rejection time and mechanism

A

< 6 months

Cellular - CD4 activating type IV reaction - cellular T cell infiltrate

Antibody - B cell activation, antibodies attack vessels - vasculitis

161
Q

Chronic transplant rejection time and risk factors

A

> 6 months

RF: miltiple acute rejections, HTN, hyperlipidaemia

(immune and non-immune mechanisms)

162
Q

GvHD time, presentation and treatment

A

days-weeks

Rash, vomiting, bloody diarrhoea, jaundice

Cyclosporin, methotrexate

163
Q

Acute vascular rejection is similar to a hyperacute reaction, but how does it differ?

A

Only after xenograft

Days - weeks

164
Q

What type of test determines HLA type?

A

PCR

165
Q

What is a crossmatch?

A

Tests if serum from recipient is able to kill donor lymphocytes - positive crossmatch is contraindication

166
Q

What medication is given before transplant?

A

Alemtuzumab (anti-CD52)
Basiliximab (anti-CD25)
OKT3

167
Q

2 steps of HSCT

A

Eliminate hosts immune system - total body irradiation; cyclophosphamide

Replace with own (autologous) or HLA-matched (allogeneic) bone marrow

168
Q

Why is there a decrease in RA symptoms during pregnancy?

A

Shift from Th1 to Th2

169
Q

Why is there a 20x increase risk of death from MI in individuals who have had a transplant?

A

Transplant and immunsuppression -> HTN and hyperlipidaemia post-transplant

Th2 effects

Increased risk of atheroscleoris

170
Q

HIV infections per day

A

5000 (>10% children)

171
Q

HIV cycle

A

RNA retrovirus
• Targets CD4+
• Gp120 (initial) and gp41 on HIV binds to CD4 and CCR5/CXCR4
• Reverse transcriptase converts RNA into DNA
• Integrase integrates viral DNA into host’s DNA
• Viral proteins created and cut with proteases

172
Q

What is the major structural protein of HIV?

A

Gag protein (group specific antigen)

> 50% mass of viral particle

173
Q

Which antibodies against HIV are neutralising and non-neutralising?

A

Neutralising - anti-GP120, anti-GP41

Non-neutralising - antip24 gag IgG

(still infectious when coated with abs)

174
Q

Which chemokines can CD8+ produce to prevent HIV entry?

A

MIP-1a
MIP-1b
RANTES

175
Q

Why are naive CD8+ not primed in HIV infection?

A

Infected CD4+ killed
Memory lost and failure to activate monocytes and dendritic cells
These then can’t prime CD8+

176
Q

How are HIV quasispecies produced?

A

Error-prone reverse transcriptase - escape immune response

177
Q

Time from HIV infection to AIDS

A

Median: 8-10 years
Rapid progressors: 2-3 years
Long term non progressers: stable CD4+ and no symptoms after 10 years

178
Q

What predicts HIV disease progression?

A

Initial viral burden

179
Q

HIV screening test

A

ELISA - detects anti-HIV antibody

180
Q

HIV confirmation test

A

Western blot - detects anti-HIV antibody

181
Q

When is the HIV test correct and why?

A

~10 weeks incubation from infection

Patient has to have seroconverted

182
Q

What testing is done following diagnosis of HIV?

A

Viral load - PCR to detect RNA
CD4 - FACS (flow cytometry)
Phenotypic resistance - viral replication measured under selective pressure of increasing concentrations of ART, compared to wild-type
Genotypic resistance - direct sequencing of amplified HIV genome

183
Q

AIDS CD4 count

A

< 200 cells/uL

184
Q

When should HIV treatment be commenced?

A

Immediately once diagnosis confirmed

old guidelines start thinking CD4 <350 and commence <200

185
Q

What does highly active anti-retroviral therapy (HAART) include?

A

2x nucleoside/tide RT inhibitors + protease inhibitor (or non-NRTI)

e.g. emtricitabine + tenofovir + efavirenz

186
Q

When should zidovudine infusion be used in women with HIV during pregnancy?

A
  • Untreated women with unknown viral load

* Considered if viral load > 50, use if over 1000 (on ART)

187
Q

HIV ART for neonates

A

Zidovudine

2 weeks if very low risk
4 weeks if low risk
(6 weeks PO on path guide)

Combination PEP if higher risk

188
Q

Why might amlodipine not work in a patient also taking efavirenz?

A

Efavirenz (NNRTI) is a CP450 inducer

Amlodipine broken down too quickly

189
Q

Maraviroc is an attachment inhibitor. What does it target?

A

Blocks CCR5

190
Q

What do the names of reverse transcriptase inhibitors, integrase inhibitors, and protease inhibitors end with respectively?

A
  • ine
  • gravir
  • avir
191
Q

When does PEP need to be taken?

A

<72 hours of exposure

Take for 4 weeks

192
Q

How long can immunologic memory last?

A

up to 65 years

193
Q

Where are effector memory cells found?

A

Liver
Lungs
GIT

194
Q

Is CCR7 and CD62L present in central and effector memory cells?

A

CCR7+ in central and effector
CD62L high in central and low in effector

Allows migration via high endothelial venules to peripheral lymph nodes

195
Q

What cytokines do central and effector memory cells produce?

A

Central - IL2

Effector - perforin, IFN-gamma

196
Q

Is there more effector memory in CD4 or CD8 population?

A

CD8

197
Q

Which immunoglobulins are involved in B cell expansion/isotope switching, stimulated by antigens?

A

IgM - T cell independent

IgG, IgA, IgE - T cell dependent

198
Q

Outline difference between Th1, Th2, and Th17 (all T helper cells) response

A

Th1
• Cell mediated
• Help APCs (e.g. macrophages) and CD8+

Th2
• Humoral
• BCR takes up antigen, presented on MHC II, Th2 binds and activates B cell

Th17
• Neutrophil recruitment
• Targets fungal and bacterial infection

199
Q

Cytokines produced in Th1, Th2, and Th17 response

A

Th1
• IL2
• IFN-gamma
• TNF

Th2
• IL4, 5, 6

Th17
• IL17, 21, 22

200
Q

Where is the vaccine antigen processed if injected IV?

A

Spleen

201
Q

How long does protection after BCG last?

A

10-15 years

202
Q

Which cells should be targeted in a cancer vaccine?

A

APCs to express tumour antigen i.e. dendritic cells

203
Q

Signs of immune senescence

A
  • Accumulation of terminally differentiated effector memory T cells
  • More senescence markers
  • Reduced ability to respond to new antigens
  • Low-grade inflamation
204
Q

What types of pathogens are conjugated vaccines used against?

A

Encapsulated bacteria

205
Q

Live attenuated vaccine benefits

A
  • Long immunity
  • Protects against cross reactive strains
  • Activates all phases of immune system
206
Q

3 examples of live attenuated vaccines

A

BCG
MMR
Typhoid

207
Q

3 examples of inactivated vaccines

A

Salk (polio)
Hep A
Pertussis

208
Q

2 examples of subunit vaccines

A

Hep B

HPV

209
Q

3 examples of conjugate vaccines

A

Hib
Meningococcus
Pneumococcus

210
Q

2 examples of toxoid vaccines

A

Diptheria

Tetanus

211
Q

Disadvantage of DNA vaccines

A

Could lead to autoimmune diseases e.g. SLE

212
Q

Can PWHIV have MMR, BCG or yellow fever vaccine?

A

Only MMR

213
Q

What is the most common vaccine adjuvant and what does it do?

A

Alum
• Slows antigen release
• Activates Gr1+ cells to produced IL4
• Helps prime naive B cells

214
Q

How does CpG adjuvant work?

A

Cystosine next to guanine in DNA

Triggers TLRs on APCs

215
Q

How does complete Freund’s adjuvant work?

A

Water-in-oil emulsion containing mycobacterial cell wall components (mainly for animals)

216
Q

How does ISCOMS (immune stimulating complex) adjuvant work?

A

Experimental

Spherical open cage-like structures formed when mixing together cholesterol, phospholipids and saponins

217
Q

Which interleukin can be used as an adjuvant?

A

IL12

218
Q

How long do passive vaccines last? What can human normal Ig (HNIG) be used for?

A

3 weeks

Hep A, measles, ITP, Kawasaki, GBS

219
Q

How is the mantoux test carried out?

A

Inject 0.1ml of 5 tuberculin units (purified protein derivative) intradermally

Examine arm 48-72 hours later

Positive result: >10mm induration (not including erythema) - means previous exposure to tuberculin

220
Q

What is the aim of the BCG vaccine?

A

Stop activation of TB
Efficacious against miliary and meningeal TB
Only some protection against pulmonary TB

Path (5 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions