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Pathology- Imperial College London MBBS > Immunology > Flashcards

Immunology Flashcards

1
Q

IgA deficiency affects what type of surface?

A

Mucosal surfaces

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2
Q

Which cell type is affected in reticular dysgenesis?

A

Neutrophils (reduced production)

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3
Q

Which mutation is associated with reticular dysgenesis?

A

Enzyme adenylate kinase 2

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4
Q

Which primary immune deficiency is defined as an autosomal recessive congenital failure of neutrophil maturation?

A

Kostmann syndrome

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5
Q

Which enzyme is affected in cyclic neutropenia?

A

Neutrophil elastase

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6
Q

What is the inheritance pattern of cyclic neutropenia?

A

Autosomal dominant episodic neutropenia every 4-6 weeks

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7
Q

Which primary immune deficiency is associated a very high neutrophil account, and delayed umbilical cord separation?

A

Leukocyte adhesion deficiency

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8
Q

Which adhesion molecules are insufficiently expressed in Leukocyte adhesion deficiency?

A

CD11a/CD18 and CD11b/CD18

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9
Q

Which neonatal complication is associated with leukocyte adhesion deficiency?

A

Delayed umbilical cord separation

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10
Q

What neutrophil pathology is observed in leukocyte adhesion deficiency?

A

Neutrophilia (raised neutrophil count)

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11
Q

Which primary immunodeficiency is characterised by deficient NADPH oxidase?

A

Chronic granulomatous disease

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12
Q

Which investigations are indicated for establishing the diagnosis of deficiency oxidative killing?

A

Nitro-Blue Tetrazolium test

and

DHR flow cytometry

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13
Q

What NBT test result is observed in chronic granulomatous disease?

A

Negative

(dye normally changes colour from yellow to blue following the interaction with hydrogen peroxide)

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14
Q

In DHR flow cytometry test, DHR is oxidised to what, following the interaction with hydrogen peroxide?

A

Rhodamine

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15
Q

What is the management for chronic granulomatous disease (NADPH oxidase deficiency)?

A

Interferon gamma

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16
Q

What enzyme is deficient in chronic granulomatous disease?

A

NADPH oxidase (oxygen is not converted to superoxide)

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17
Q

In chronic granulomatous disease (NADPH oxidase deficiency), which types of organisms proliferate?

A

Catalase positive bacteria

PLACES
Pseudomonas
Listeria
Aspergillus (mould)
Candida (fungus)
Staph aureus
Serratia

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18
Q

Deficiency of which interleukin predisposes individuals to mycobacteria?

A

IL-12 and IFN-y (Inability to form granulomas - as infected macrophages typically produce IL12)

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19
Q

Which factors are associated with the alterantive pathway?

A

Factor B, D, P (properdin)

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20
Q

Alternative pathway complement deficiencies predisposes the host to which type of bacteria?

A

Encapsulated organisms

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21
Q

What role does properdin have in the complement cascade?

A

Stabilises C3 convertase - triggers the membrane attack complex (MAC

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22
Q

Classical pathway complement deficiency is associated with which type III hypersensitivity disorder?

A

SLE

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23
Q

Which complement (C) is the divergence point for all complement pathways?

A

C3

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24
Q

C3 deficiency predisposes the host to recurrent infected by which type of bacteria (Name 3)?

A

(esp. encapsulated – meningococcus, streptococcus, haemophiles)

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25
Q

Secondary C3 deficiency is associated with what type of factors?

A

Nephritic factors - C3 auto-antibodies

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26
Q

X-linked SCID is associated with which mutation?

A

Gamma-chain of IL2 receptor

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27
Q

What is the observed phenotype for X-linked SCID?

A

Very low or absent T and NK cell

Normal or increased B cells

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28
Q

What role does IL-2 receptors have?

A

Respond to cytokines to promote T cell and NK cell development

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29
Q

What is the phenotype observed in adenosine deaminase deficiency?

A
  • Very low or absent T cell and NK cell numbers
  • Very low or absent B cell numbers
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30
Q

What role does adenosine deaminase have?

A

Lymphocytes require ADA for cell metabolism

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31
Q

Which mutation is associated with DiGeorge Syndrome?

A

TBX1

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32
Q

Which chromosomal aberration is associated with DiGeorge syndrome?

A

22q11.2.

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33
Q

What is the diagnosis for the following features:

  • Cardiac abnormalities (especially tetralogy of Fallot)
  • Abnormal facies (high forehead, low set ears)
  • Thymic aplasia (T cell lymphopenia)
  • Cleft palate
    *Hypocalcaemia/hypoparathyroidism
A

DiGeorge syndrome
(22q11.2 deletion syndrome)

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34
Q

What is the developmental defect associated with DiGeorge Syndrome regarding primary immune deficiency?

A

Thymic aplasia

Pharyngeal pouch

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35
Q

What are the clinical manifestations associated with DiGeorge syndrome
(22q11.2 deletion syndrome)?

A

Remember CATCH-22:
* Cardiac abnormalities (especially tetralogy of Fallot)
* Abnormal facies (high forehead, low set ears)
* Thymic aplasia (T cell lymphopenia)
* Cleft palate
* Hypocalcaemia/hypoparathyroidism
* 22 – chromosome (Detected by FISH cytogenetic analysis).

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36
Q

What is the B/T cell phenotype observed in DiGeorge Syndrome?

A

Normal number of B cells

Reduced numbers of T cells

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37
Q

Bare lymphocyte syndrome is associated an absent expression of which molecule?

A

MHC-II molecules

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38
Q

Which T cell is deficient in bare lymphocyte syndrome Type II?

A

CD4+ cells

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39
Q

CD40L deficiency is associated with which type of primary immune deficiency?

A

Hyper-IgM syndrome

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40
Q

Which CD ligand is deficient in Hyper IgM syndrome?

A

CD40L

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41
Q

What is the inheritance pattern for Wiskott-Aldrich Syndrome ?

A

X-linked recessive

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42
Q

Which cell type is affected in Bruton’s X-linked hypogammaglobulinaemia?

A

B-cells
(Pre-B cells cannot develop to mature B cells)

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43
Q

Which enzyme is defective in Bruton’s X-linked hypogammaglobulinaemia?

A

Tyrosine kinase

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44
Q

CD40L mutation results in what primary immune deficiency?

A

Hyper IgM syndrome

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45
Q

What effect does HyperIgM syndrome have on immunoglobulins?

A

Elevated Serum IgM

Undetectable IgA, IgE, IgG

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46
Q

Which primary immune deficiency is characterised by a marked reduction in IgG with low IgA and IgM?

A

Common variable immune deficiency

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47
Q

In a DHR flow cytometry test, what is the positive reaction?

A

Oxidised to rhodamine – strongly fluorescent following interaction with hydrogen peroxide.

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48
Q

What are the positive responses for a NBT test?

A
  • Nitro-Blue Tetrazolium test
  • Dye that changes colour from yellow to blue, following interaction with hydrogen peroxide.
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49
Q

What are the antibiotic prophylaxis for immunodeficiency?

A

Septrin (co-trimoxazole)

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50
Q

What is the definitive therapy for phagocyte deficiency?

A

Haematopoietic stem cell transplantation

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51
Q

What are the diagnostic tests for complement deficiency?

A

CH50 (classical pathway)

AP50 (Alternative pathway)

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52
Q

Which interleukins are responsible for causing fever?

A

IL-1, and IL-6

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53
Q

Which cytokines are implicated in monogenic auto-inflammatory disease?

A

TNF-alpha and IL-1

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54
Q

What is the inheritance pattern for Familial Mediterranean Fever?

A

Autosomal recessive

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55
Q

Which gene is implicated in Familial Mediterranean Fever?

A

MEFV

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56
Q

Which enzyme is raised in Familial Mediterranean Fever?

A

Pro-caspase 1 - increases TNF-alpha and IL-1

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57
Q

What is the clinical presentation for Familial Mediterranean Fever?

A

Clinical Presentation – periodic fevers lasting 48-96 hours, associated with:
* Abdominal pain (peritonitis)
* Chest pain (pleurisy and pericarditis)
* Arthritis
* Rash

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58
Q

What is the long term risk associated with Familial Mediterranean Fever?

A

AA amyloidosis

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59
Q

Why is the risk of AA amyloidosis increased in Familial Mediterranean Fever?

A

Liver produces serum amyloid AA as an acute phase protein - deposition in kidneys, liver and spleen = nephrotic syndrome (proteinuria) + renal failure.

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60
Q

AL amyloidosis is associated with what?

A

Multiple myeloma

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61
Q

What is the medical management for Familial Mediterranean Fever?

A

Colchicine

Anakinra

Etanercept

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62
Q

What is the mechanism of action for Colchicine?

A

Binds to tubulin in neutrophils - disrupts function in migration/cytokine

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63
Q

What is the mechanism of action of anakinra?

A

IL-receptor antagonist

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64
Q

What is the mechanism of action for etanercept?

A

TNF-alpha inhibitor

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65
Q

Name a TNF-alpha inhibitor

A

Etanercept

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66
Q

Name 2 pattern recognition receptors?

A

Toll-like receptors and mannose receptors

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67
Q

Antibodies bind to which type of receptors?

A

Fc receptors

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68
Q

Which enzyme is implicated in oxidative killing?

A

NADPH oxdiase

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69
Q

NADPH oxidase complex converts oxygen to which type of reactive oxygen species?

A

Superoxide and hydrogen peroxide

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70
Q

Which bactericidal enzymes are implicated in non-oxidative killing of bacteria in phagolysome?

A

lysozyme and lactoferrin

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71
Q

What is the site of T-cell maturation?

A

Thymus

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72
Q

What is the site of B cell maturation?

A

Bone marrow

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73
Q

Name 3 secondary lymphoid organs

A

Lymph nodes
Spleen
Mucosal-associated lymphoid tissue (MALT)

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74
Q

What role does the thymus play in T-cell maturation?

A

Positive and negative selection

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75
Q

Which CD factor is expressed by all T-cells?

A

CD3

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76
Q

CD8+ bind to which MHC molecule?

A

MHC-I

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77
Q

CD4+ binds to which MHC molecule?

A

MHC-II

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78
Q

Role of Th1 cells?

A

Helps CD8 and macrophages produce IL-2, IFN-Y and TNF.

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79
Q

Role of Th2 cells?

A

Humoral response - Helper T cell produce IL-4,5,6

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80
Q

Which cell is recruited by Th17 cells?

A

Neutrophils

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81
Q

Which IL is secreted by Th17 cells (3 types)?

A

IL-17, 21, 22

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82
Q

What is expressed by T regulatory cells?

A

CD25 and FoxP3

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83
Q

Which type of T cells promote germinal centre reactions and B-cell differentiation

A

T follicular helper cells

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84
Q

Which factors and enzymes are released by CD8 T cells?

A

Perforin
Granzymes

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85
Q

Which cytokines are secreted by CD8 T-cells?

A

TNF-alpha and IFN-gamma

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86
Q

Which is the first type of immunoglobulin is produced by B-cells?

A

IgM

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87
Q

Which ligand interaction supports B-cell differentiation?

A

CD40L:CD40 interaction

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88
Q

What two processes do B-cells undergo?

A

Isotype switching
Somatic hypermutation

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89
Q

Which antibody region is the variable region?

A

FAB - light chains

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90
Q

Where are central memory cells made?

A

High endothelial venules (HEVs)

Lymph nodes

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91
Q

What protein is used for the Mantoux test?

A

Purified protein derivative

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92
Q

Threshold for positive mantoux test?

A

> 10 mm in diameter

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93
Q

Positive NBT test colour?

A

Blue

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94
Q

Negative NBT test colour?

A

Yellow

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95
Q

Which IL causes fever?

A

IL-1, IL-6

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96
Q

Clinical presentation of familial mediterranean fever?

A

Clinical Presentation – periodic fevers lasting 48-96 hours, associated with:
* Abdominal pain (peritonitis)
* Chest pain (pleurisy and pericarditis)
* Arthritis
* Rash

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97
Q

Which gene is implicated in Crohn’s disease?

A

NOD2 and iBD1 gene.

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98
Q

What doses NOD2 code for?

A

Cytoplasmic microbial sensor

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99
Q

What is the management for Crohns disease?

A

Corticosteroid
Anti-TNF-alpha antibodies

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100
Q

Failure of central tolerance is associated with a defect in what gene?

A

Autoimmune regulator gene (AIRE)

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101
Q

Failure of peripheral tolerance is associated with which gene?

A

Foxp3

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102
Q

Molecular self-mimicry is associated with what condition?

A

Rheumatic fever
Ankylosing spodylitis

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103
Q

Name a monogenic autoimmune disease of central tolerance?

A

Autoimmune polyendocrine syndrome type 1 (APS1)

Auto-immune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED)

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104
Q

What is the inheritance pattern for autoimmune polyendocrine syndrome type 1?

A

Autosomal recessive

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105
Q

Which endocrine autoimmune disorders are associated with APS1?

A
  • Multiple auto-immune endocrine diseases: Hypothyroidism, Hypoparathyroidism (85%), Addison’s (78%), Diabetes, Vitiligo, candidiasis (98% - anti-IL17 and anti-IL22 antibodies)
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106
Q

Mutations in which gene is associated with IPEX?

A

Foxp3

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107
Q

Name a monogenic autoimmune disease associated with peripheral tolerance?

A

Immune dysregulation polyendocrinopathy enteropathy (IPEX)

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108
Q

what are the clinical features associated with IPEX?

A

Three Ds - diabetes mellitus, diarrhoea, dermatitis

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109
Q

Which cells are affected in IPEX (peripheral tolerance defect)?

A

Treg cells

Autoreactive B cells

Failure to negatively regulate T cells

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110
Q

What is the inheritance pattern for ALPS?

A

Autosomal dominant

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111
Q

Goodpasture disease - allele

A

HLA-DR15

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112
Q

Graves disease - allele

A

HLA-DR3

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113
Q

HLA allele in rheumatoid arthritis

A

HLA-DR4

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114
Q

Which antibody is associated with bullous pemiphigoid?

A

Anti-hemidesmosome

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115
Q

Coeliac disease -antibody

A

Coeliac disease

Anti-tissue transglutaminase antibody (IgA), Anti-endomysial antibody (IgA)
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116
Q

Neonatal complication is the context of Anti-Ro antibody (to mothers with SLE)?

A

Congenital heart block

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117
Q

Which antibody in SLE is associated with congenital heart block?

A

Anti-Ro

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118
Q

Which antibody is implicated in dermatitis herpetiformis?

A

Anti-endomysial antibodies

119
Q

Goodpasture’s antibody

A

Anti-GBM antibody

120
Q

Graves disease is associated with with autoantibody?

A

TSH-receptor antibody

121
Q

Hashimoto’s thyroiditis is associated with which two autoantibodies?

A

Antibodies to Thyroglobulin and Thyroperoxidase

122
Q

Anti-Ach Receptor Antibody is associated with what autoimmune disease?

A

Myasthenia Gravis

123
Q

Autoantibodies associated with pernicious anaemia?

A

AB to gastric parietal cells
Intrinsic factor

124
Q

Anti-mitochondrial antibody is associated with which autoimmune condition?

A

Primary biliary cirrhosis

125
Q

Antibodies to glutamase decarboxylase is associated with which disease?

126
Q

Which autoantibodies is associated with autoimmune TTP?

A

Anti-Glycoprotein IIb-IIIa or Ib-IX Antibody

127
Q

Which specific autoantibody is implicated in rheumatoid arthritis?

128
Q

Which joint is spared rheumatoid arthritis?

A

Distal interphalangeal joints

129
Q

What are the hand deformities associated with rheumatoid arthritis?

A

Swann neck, Boutinnier’s, Z-thumb and ulnar deviation

130
Q

Which autoantibody is associated with SLE?

A

Anti-dsDNA antibody

131
Q

Which type of rash is associated with SLE?

A

Malar rash

132
Q

Anti-Ro and Anti-La autoantibodies are associated with which autoimmune disorder?

A

Sjogren’s disease

133
Q

Dermatomyositis is associated with which autoantibody?

A

Anti-Jo antibody

134
Q

Which characteristic rash is associated with Dermatomyositis?

A

Heliotrope rash

135
Q

Gottron’s papules are associated with which autoimmune disorder?

A

Dermatomyositis

136
Q

What is the characteristics of polymyositis?

A

Respiratory weakness
Diffuse myopathy
Dysphagia

137
Q

Which enzyme is elevated in Dermatomyositis ?

A

Creatine kinase

138
Q

CREST is associated with which autoantibodies?

A

Anti-centromere

139
Q

What are the features associated with CREST?

A
  • Calcinosis
  • Raynaud’s
  • Oesophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
140
Q

Anti-Scl70 antibodies are associated with what autoimmune disorder?

A

Diffuse CREST

141
Q

cANCA is directed against which enzyme?

A

Proteinase 3

142
Q

Granulomatosis with Polyangiitis
(Wegner’s) is associated with which type of antibody?

143
Q

pANCA is associated with which type of polyangitis?

A

Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss)

Microscopic polyangitis

144
Q

pANCA is directed against which enzyme?

A

Myeloperoxidase

145
Q

Allergic asthma is associated with which polyangitis?

A

Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss)

146
Q

Which acute post-transfusion reaction is associated with a transient rise in temperature?

A

Febrile. non-haemolytic transfusion reaction

147
Q

What is the management for febrile non-haemolytic transfusion reaction?

A

Paracetamol - stop/taper transfusion

148
Q

Allergic transfusion reaction is associated with what presentation?

A

Mild urticarial rash + pruritus accompanied by heeze caused by allergy to donor plasma proteins

149
Q

What is the management for allergic transfusion reactions?

A

IV antihistamines

150
Q

ABO incompatibility is mediated by what Immunoglobulin?

151
Q

What is the primary cause of ABO incompatibility post-transfusion?

A

Failure of bedside check

152
Q

Which blood product is susceptible to bacterial contamination?

A

Platelets (stored at room temperature)

153
Q

Which patients are at an increased risk of anaphylaxis post-transfusion?

A

Patients with IgA deficiency

154
Q

What two pulmonary post-transfusion reactions are severe?

A

Transfusion-associated circulatory overload

Transfusion-related acute lung injury

155
Q

Which drug is indicated in the management of TACO?

156
Q

Which pulmonary acute transfusion reaction is associated with fever?

A

Transfusion-related acute lung injury

157
Q

What are the clinical features of TRALI?

A

Dyspnoea, low oxygen saturations, fever, high HR, high BP.

158
Q

What CXR findings are implicated in TRALI?

A

Bilateral pulmonary infiltrates during/within 6 hours of transfusion due to circulatory overload.

159
Q

Which antibodies are implicated in the pathophysiology of TRALI?

A

Anti-WBC antibodies in donor blood - aggregate to pulmonary capillaries and release neutrophil proteolytic enzymes

160
Q

Which two antigens are implicated in delayed haemolytic transfusion reaction?

A

Duffy and Kidd

161
Q

What prophylactic measure is implicated in mitigating CMV post-transfusion infection

A

Leucodepletion/irradiation

162
Q

What is the consequence of parvovirus infection post-transfusion?

A

Red cell aplasia

163
Q

What are the consequences of transfusion-associated graft versus host disease?

A

Diarrhoea, liver failure, skin desquamation, bone marrow failure, death

164
Q

Which antigen is implicated in the pathophysiology of post-transfusion purpura?

A

Human platelet antigen 1a negative patients

165
Q

Which test checks for ffDNA in maternal circulation?

A
  • Kleihauer test
166
Q

Which Ig is implicated in the pathophysiology of Type 1 hypersensitivity disorders?

167
Q

Mast cell degranulation in T1HD results in the release of what?

A

histamine, proteases, prostaglandin, cytokines, platelet activating factors and leukotrienes.

168
Q

What are the physiological effects of mast cell degraulation?

A
  • Increased vascular permeability.
  • Peripheral vasodilation
  • Smooth muscle contraction – increased mucous secretions, bronchospasms, abdominal cramping.
  • Rhinitis
169
Q

How is a diagnostic of food allergy made?

A

Food diary and skin prick testing

170
Q

Diagnostic test for latex food syndrome?

A

Skin prick testing

171
Q

Which is deficient in hereditary angioedema?

A

C1 inhibitor

172
Q

What is used as a positive control for skin prick testing?

173
Q

How long should anti-histamines be discontinued for prior to skin prick testing?

174
Q

Which allergen is associated with peanuts and nuts?

175
Q

Ara h8 protein is associated with what?

A

Oral reactions to peanut and stone fruit

176
Q

What quantitative test is used to measure IgE in serum>

177
Q

What is the diagnostic test to food allergens?

A

Challenge test

178
Q

During an acute allergic reaction, what should be measured?

A

Measure mast cell tryptase

179
Q

What type of hypersensitivity reaction is characterised by auto-antibody production?

180
Q

Evan’s syndrome is characterised by what?

A

ITP + AIHA

181
Q

Autoimmune thrombocytopenia purpura is associated with which antigen?

A

Glycoprotein iib, and iiia

182
Q

What hypersensitivity reaction is Goodpasture’s syndrome?

183
Q

What is the antigen implicated in goodpasture’s syndrome?

A

Collage type IV

184
Q

Which antibody is associated with Goodpasture’s syndrome?

A

Anti-GBM antibody

185
Q

What is the histological appearance of goodpasture’s syndrome?

A

Linear smooth deposition of IgG deposits on the basement membrane

186
Q

What is the antigen in pemphigus vulgaris?

A

Desmoglein-3

187
Q

What antibody is associated with Myasthenia gravis?

A

Anti-Acetylcholine receptor

188
Q

What is the diagnostic tests implicated with Myasthenia Gravis?

A

Tensilon test and EMG

189
Q

What type of hypersensitivity reaction is acute rheumatic fever?

190
Q

Which criteria is used to diagnose Acute rheumatic fever?

A

Jones Criteria

191
Q

Churg-Strauss Syndrome is associated with which antibody?

192
Q

p-ANCA is targeted against what?

A

myeloperoxidase

193
Q

Wegener’s Granulomatosis
(GPA)
- antibody?

194
Q

c-ANCA target?

A

Proteinase 3

195
Q

Serum sickness is which type of hypersensitivity?

196
Q

Which antigen is implicated in serum sickness?

A

Reaction to Proteins in Antiserum (Penicillin)

197
Q

What angiographic sign is seen with polyarteritis nodosa?

A

Rosary-bead/pearl sign

198
Q

Which antibody is associated with drug-induced SLE?

A

Anti-histone

199
Q

What is the most specific antibody for SLE?

200
Q

C1q deficiency is associated with which type 3 hypersensitivity reaction?

201
Q

Contact dermatitis and tubercilin-type hypersensitivity is what type of hypersensitivity reaction?

202
Q

Which cell type mediates a type IV hypersensitivity reaction?

A

T-cell mediated

203
Q

What forms part of an intradermal TB injection?

A

Purified protein derivative

204
Q

Which antigen is implicated in type 1 DM?

A

Glutamate Decarboxylase

205
Q

Antigen in multiple sclerosis?

A

Myelin basic protein

206
Q

CSF finding for MS?

A

Oligoclonal bands

207
Q

What is the most specific auto-antibody for RA?

208
Q

Prednisolone inhibits which enzyme?

A

phospholipase A2

209
Q

BK virus causes what?

A

Haemorrhagic cystitis

210
Q

Mycophenolate mofetil inhibits what?

A

Anti-metabolic, inhibits IM PDH - inhibits guanine synthesis

211
Q

Mycophenolate mofetil can activate which virus?

A

John Cunningham virus

212
Q

Reaction of the JC virus can result in what?

A

progressive multifocal leukoencephalopathy

213
Q

What is the active metabolite of azathioprine?

A

6-mercaptopurine

214
Q

What should be checked prior to azathioprine therapy?

215
Q

Methotrexate inhibits which enzyme?

A

Dihydrofolate reductase

216
Q

What is the management option for severe Goodpasture disease and type-ii disease?

A

Plasmapheresis

217
Q

What can be administered to reduce ABO incompatibility and AB-mediated transplant rejection?

A

Plasmapheresis

218
Q

Tacrolimus is what class of drug?

A

Calcineurin inhibitor

219
Q

Calcineurin inhibitors e.g., tacrolimus and cyclosporin reduce what IL-expression?

220
Q

What are the complications associated with calcineurin inhibitors?

A

Nephrotoxic, hypertension, neurotoxic, dysmorphism, gingival (gum) hypertrophy

221
Q

Tofacitinib inhibits what?

A

JAK1/3 inhibitor

222
Q

Apremilast inhibits which enzyme?

223
Q

Name an Anti-CD25 inhibitor indicated in prophylaxis for allograft rejection?

A

Basiliximab

224
Q

What does Basiliximab target?

A

Anti-CD25 - alpha chain of iL-2 - inhibits t-cell proliferation

225
Q

Abatacept is implicated in the management of which disease?

A

Rheumatoid arthritis

226
Q

What is the target for Abatacept?

A

Anti-CTLA4-Ig fusion protein

227
Q

Rituximab is indicated for which conditions?

A

Rheumatoid arthritis, lymphoma, SLE

228
Q

What is the target for rituximab?

229
Q

Which monoclonal antibody is implicated in the management for severe inflammatory bowel disease?

A

Vedolizumab

230
Q

Vedolizumab targets what?

A

Anti-alpha-4-beta-7 intergrin

231
Q

Which monoclonal antibody targets alpha-4-beta-10-intergrin and is used in Crohn’s disease?

A

Natalizumab

232
Q

What is the target for Tocilizumab?

A

Anti-IL-6 Receptor

233
Q

Tocilizumab is used to treat which 2 conditions?

A

Rheumatoid arthritis and Castleman’s disease

234
Q

Muromonab targets what?

A

CD3 on T-cells

235
Q

Name 2 anti-TNF-alpha drugs

A

Infliximab, adalimumab

236
Q

Etancerpt targets what?

A

TNF-alpha receptor

237
Q

Which drug is an anti-iL17?

A

Secukinumab

238
Q

Secukinumab is used to treat what?

A

psoriasis, psoriatic arthritis, ankylosing spondylitis

239
Q

Which term describes a transplant from a twin?

240
Q

Which term describes a transplant from the same species?

241
Q

Which term describes a transplant between two difference species?

242
Q

Name 3 types of HLA Class 1 expressed by all cells?

243
Q

Which cells present MHC-II?

A

CD4+ Cells

244
Q

Which HLA antigen is the most important to mitigate mismatch?

245
Q

The effector phase of T-cell mediated graft rejection involves which type of T-cells and ligand?

A
  • Cytotoxic T cells – Release of toxins (granzyme B), punch homes in target cells (perforin), apoptotic cell death (Fas ligand).
246
Q

What are the histological features associated with T-cell mediated rejection?

A

Histological Features:
* Lymphocytic interstitial infiltration
* Ruptured tubular basement membrane.
* Tubulitis (inflammatory cells within the tubular epithelium).
* Macrophages, recruited by the T-cells.

247
Q

Antibody-mediated rejection is associated with which cell type?

A

Endothelium - intra-vascular disease

248
Q

What can prevent hyperacute graft rejection?

A

Crossmatch ABO groups and HLA-matching

249
Q

What is the maximum HLA mismatches for a parent-child transplant?

250
Q

What three HLAs are matched?

A

HLA-A, HLA-B, HLA-DR

251
Q

what are the three types of assays to assess for cross-reactivity in transplant?

A
  1. Cytotoxicity/Complement dependent cytotoxicity – Does recipient serum kill donor lymphocytes.
  2. FACS/Flow cytometry – Does recipient serum bind to donor lymphocytes.
  3. Solid phase/Luminex – Does recipient serum contain antibodies to individual HLA molecules.
252
Q

What induction immunosuppressive regimen is used pre-transplant?

A

Anti-CD52 Alemtuzumab or anti-CD25 basiliximab or OKT3/ATG.

253
Q

Name a proteasome inhibitor?

A

bortezomib

254
Q

What drugs are used for the maintenance therapy post-transplant?

A

Steroid + calcineurin inhibitor + antiproliferative agent (azathioprine/mycophenolate mofetil).

255
Q

What are the symptoms of graft-versus-host disease?

A
  • Symptoms: Skin desquamation, rash, GI disturbance (nausea, vomiting, abdominal pain, diarrhoea, bloody stool), liver failure (jaundice), BM failure.
256
Q

Which drug is the prophylaxis for GVHD?

A

Methotrexate/cyclosporine, irradiate blood components for immunosuppressed patients.

257
Q

What is the management for GVHD?

A

Corticosteroids

258
Q

Which opportunistic infections are increased post-transplant?

A

CMV, BK virus, Pneumocystis carinii.

259
Q

Kaposi-sarcoma is associated with which virus?

260
Q

Which cardiovascular risk is increased post-transplant?

A

Myocardial infarction secondary to atherosclerosis

261
Q

What antibodies are associated with Sjogren’s syndrome?

A

Anti-Ro and Anti-La

262
Q

Which sex is affected the most in Sjogren’s syndrome?

A

Female predominance 9:1

263
Q

What are the characteristic features of Sjogren’s disease?

A
  • Dry eyes – keratoconjunctivitis sicca, corneal ulcers, keratitis.
  • Dry mouth – increased caries
  • Major salivary gland swelling – parotid submandibular.
  • Extra glandular manifestations – Fatigue, arthritis, arthralgia/myalgia
  • Pulmonary – interstitial lung disease
264
Q

Which test is used to diagnose Sjogren;s syndrome?

A

Schirmer test

Positive Rose Bengal testing

265
Q

Which HLA-haplotype is associated with ankylosing spondylitis?

266
Q

Which haplotype is associated with coeliac disease?

A

DQ2 and DQ8 HLA

267
Q

What histological findings are associated with coeliac disease?

A

villous atrophy, crypt hyperplasia and increased intra-epithelial lymphocytes.

268
Q

Derm manifestation associated with Coeliac disease?

A
  • Dermatitis herpetiformis
269
Q

Which IgA is most sensitive in coelaic disease?

A
  • IgA TGT (anti-transglutaminase antibody) ~95% specific, 90-94% sensitive
270
Q

Herd immunity threshold calculation?

A

Herd Immunity Threshold=1-1/R0

271
Q

Name a Pattern recognition receptor?

A

Toll-like receptors, retinoic acid-inducible gene 1-like receptors, NLRs.

272
Q

Influenza and polio (IPV) are what type of vaccines?

A

Inactivated

273
Q

MMR and yellow fever are what type of vaccine?

A

Live attenuated

274
Q

Name 2 toxoid vaccines?

A

Diptheria and tetanus

275
Q

What type of vaccine are NHS?

276
Q

Which vaccines are administered at 2 months?

A

DTaP/IPV/HiB/Hep B
(6 in 1 injection)
oral rotavirus
Men B

277
Q

Which 3 vaccines are administered at 3 months?

A

DTaP/IPV/HiB/Hep B
(6 in 1 injection)
Oral rotavirus
PCV

278
Q

When is the MMR administered for the first time?

A

12 months (second is 3 years 4 months)

279
Q

Which 2 vaccines are given during pregnancy?

A

Influenza and DtAP/IPV - pertussis

280
Q

Name 6 additional travel vaccinations

A

Cholera, Hepa A, HepB, Jap encephalitis, Tick-borne encephalitis, Typhoid, Yellow Fever.

281
Q

Which vaccine is given after 70-years of age?

282
Q

When is the PPV vaccine given?

A

65-years of age

283
Q

Which 2 vaccines can HIV positive patients not receive?

A

BCG and yellow fever

284
Q

Which is a commonly used depot adjuvant?

285
Q

Which is the most commonly used stimulatory adjunct?

286
Q

Which interferon is given as cytokine therapy in hepatitis B and C?

A
  • Interferon alpha
287
Q

ribavirin is used to treat what?

A

Hepatitis C

288
Q

Which monoclonal antibody is used to treat advanced melanoma?

A
  • Ipilimumab
289
Q
  • Ipilimumab targets what?
290
Q

What is the target for Pembrolizumab/Nivolumab?

291
Q

What CD marker on T-cells are responsible for co-stimulation?

292
Q

JCV infects which type of cell?

A

oligodendrocytes

293
Q

Which virus is a common cause of graft loss in renal transplant patients?

Pathology- Imperial College London MBBS (5 decks)

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