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Immunology Flashcards

1
Q

What are SPUR infections?

A

Serious infections
Persistent infections
Unusual infections
Recurrent infections

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2
Q

What would make you think a patient had a serious infection?

A

If they were unresponsive to oral antibiotics

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3
Q

What two things would make you suspect persistent infections in a patient?

A
  1. Early structural damage

2. Chronic infections

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4
Q

What two things would make you think a patient had an unusual infection?

A
  1. Unusualy organisms

2. Unusual sites

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5
Q

What would you class as recurrent infections?

A

Two major or one major and recurrent minor infections in one year

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6
Q

Give 6 features that may be suggestive of primary immune deficiency

A
  1. Weight loss or failure to thrive
  2. Severe skin rash (eczema)
  3. Chronic diarrhoea
  4. Mouth ulceration
  5. Unusual autoimmune disease
  6. Family history
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7
Q

What can immunedeficiencies be classed into?

A

Secondary and primary

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8
Q

What type of immunodeficiencies are common, often subtle and often involve more than one component of the immune system?

A

Secondary

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9
Q

What type of immunodeficiencies are rare (1:10,000 live births)?

A

Primary

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10
Q

Give 5 conditions associated with secondary immune deficiency

A
  1. Physiological immune deificency
  2. Infection
  3. Treatment interventions
  4. Malignancy
  5. Biochemical and nutritional disorders
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11
Q

What two things can lead to physiological immune deficiency?

A

Ageing and prematurity

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12
Q

What are HIV (human immunodeficiency virus) and measles associated with?

A

Secondary immune deficiency (infection)

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13
Q

What three treatment interventions can lead to secondary immune deficiency?

A
  1. Immunosuppressive therapy
  2. Anti-cancer agents
  3. Corticosteroids
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14
Q

What 2 malignancies are associated with secondary immune deficiency?

A

Cancer of immune system - lymphoma, leukaemia, myeloma

Metastatic tumours

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15
Q

Give 4 biochemical and nutritional disorders associated with secondary immune deficiency

A
  1. Malnutrition
  2. Renal insufficiency/dialysis
  3. Type I and type II diabetes
  4. Specific mineral deficiencies e.g. iron and zinc
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16
Q

Name 4 innate immune system cells

A
  1. Macrophages
  2. Neutrophils
  3. Mast cells
  4. Natural killer cells
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17
Q

Name 3 innate immune system proteins

A
  1. Complement
  2. Acute phase proteins
  3. Cytokines
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18
Q

Name two cells and one protein of the acquired immune system

A

T lymphocytes and B lymphocytes

Antibody

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19
Q

What are macrophages and neutrophils?

A

Phagocytes

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20
Q

What cells: initiate and amplify the inflammatory response, sscavenge cellular and infectious debris, ingest and kill microorganisms, produce inflammatory molecules which regulate other components of the immuen system and resolution and repair?

A

Phagocytes - macrophages/monocytes and neutrophils

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21
Q

What is the main clinical feature of phagocyte deficiencies?

A

Recurrent infections

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22
Q

Name three bacteria which can give recurrent infections in phagocyte deficient patients

A
  1. Staphylococcus aureus
  2. Burkholderia cepacia
  3. Mycobacteria both TB and atypical
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23
Q

Name two fungi which can cause recurrent infections in phagocyte deficient patients

A

Candida

Aspergillus

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24
Q

What disease is treated by chemotherapy or radiotherapy that kills bone-marrow derived cells, including neutrophils?

A

Leukaemia

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25
Q

Where are neutrophil precursors initially located?

A

In the bone

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26
Q

What three problems can cause phagocyte deficiency?

A
  1. Defects of phagocyte production
  2. Defects of phagocyte mobilisation
  3. Defects of phagocyte recruitment
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27
Q

What does failure of stem cells to differentiate along myeloid lineage lead to?

A

Failure to produce neutrophils

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28
Q

Name a primary and secondary defect for failure of stem cells to differentiate along myeloid lineage

A

Primary defect: recticular dysgenesis

Secondary defect: after stem cell transplantation

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29
Q

What can specific failure of neutrophil maturation lead to?

A

Failure to produce neutrophils

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30
Q

Give two diseases associated with specific failure of neutrophil maturation

A

Kostmann syndrome: severe congenital neutropaenia

Cyclic neutropaenia: episodic neutropaenia every 4-6 weeks

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31
Q

What two features can ultimately lead to failure to produce neutrophils?

A
  1. Failure of stem cells to differentiate along myeloid lineage
  2. Specific failure of neutrophil maturation
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32
Q

At what point in the leukocyte lineage does recticular dysgenesis occur?

A

Before hematopoietic stem cell differentiation into common myeloid progenitor and common lymphoid progenitor

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33
Q

At what point of the leukocyte lineage does Kostmann syndrome occur?

A

Between the differentiation of granulocyte-monocyte progenitor into neutrophil

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34
Q

What is Kostmann syndrome?

A

A rare autosomal recessive disorder, severe chronic neutropenia

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35
Q

What is the normal neutrophil count, and what is the neutrophil count in a patient with Kostmann syndrome?

A

Normal > 3000/uL

Kostmann syndrome < 200/uL

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36
Q

How do babies with Kostmann syndrome clinically present?

A

Infections, usually within 2 weeks after birth:

Recurrent bacterial infection and systemic or localised infection

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37
Q

Name 4 non specific features of Kostmann syndrome

A
  1. Fever
  2. Irritability
  3. Oral ulceration
  4. Failure to thrive
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38
Q

What are 2 supportive treatments for Kostmann syndrome?

A

Prophylactic antibiotics

Prophylactic antifungals

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39
Q

Give two definitive treatments for Kostmann syndrome

A
  1. Stem cell transplantation - defect is in the neutrophil precursor, so strategy is to replace all precursors with allogeneic stem cells and start again
  2. Granulocyte colony stimulating factor (G-CSF) - give specific growth factor to assist maturation of neutrophils
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40
Q

What would you expect to happen if a patients phagocytes were unable to recognise endothelial adhesion molecules?

A

Leukocyte adhesion deficiency

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41
Q

Give to general statements about leukocyte adhesion deficiency

A
  1. Failure to recognise activation markers expressed on endothelial cells
  2. Neutrophils are mobilised, but cannot exit bloodstream
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42
Q

Give a clinical feature of leukocyte adhesion deficiency

A

Recurrent bacterial and fungal infections

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43
Q

What is the blood count like in leukocyte adhesion deficiency?

A

Very high neutrophil counts

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44
Q

Where is the site of infection in leukocyte adhesion deficiency?

A

Deep tissues, N.B. no pus formation

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45
Q

What type of immunodeficiency is leukocyte adhesion deficiency?

A

Rare primary immunodeficiency

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46
Q

What type of genetic defect causes leukocyte adhesion deficiency?

A

Genetic defect in leuococyte integrins (CD18)

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47
Q

What does leucocyte adhesion deficiency result in?

A

Failure of neutrophil adhesion and migration

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48
Q

Name 3 direct recognition methods of the binding of a pathogen to a macrophage

A
  1. Lectin receptors
  2. TLRs
  3. Scavenger receptors
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49
Q

Name 2 microbial-specific structures that pathogen recognition receptors recognise

A
  1. Bacterial sugars

2. Lipopolysaccharide

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50
Q

What do pathogen recognition receptors exhibit?

A

Genetic polymorphism - some are associated with increased susceptibility to bacterial infection, but most do not cause significant disease

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51
Q

What is the name for molecules that act as binding enhancers for the process of phagocytosis?

A

Opsonins

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52
Q

Name 3 opsonins

A
  1. Complement C3b
  2. IgG
  3. C-reactive protein
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53
Q

Where do opsonins bind to?

A

Receptors on phagocyte surface

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54
Q

What do Fc receptors of phagocytes allow?

A

Binding of antibody that is also bound to antigen

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55
Q

What do phagocytes express which binds to complement fragments which are also bound to antigens?

A

Complement receptor 1 (CR1)

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56
Q

What may cause defective phagocytosis?

A

Defect in opsonin receptors

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57
Q

What type of defect will also result in decreased efficiency of opsonisation, as well as defect in opsonin receptors?

A

Any defect of complement or antibody production

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58
Q

When the complement or antibody production have defects, what type of defect of recognition is this classed as?

A

Functional defect of phagocytosis

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59
Q

What type of failure is involved in chronic granulomatous disease?

A

Failure of oxidative killing mechanisms

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60
Q

What three features lead to absent respiratory burst, which leads to failure of oxidative killing mechanisms?

A
  1. Deficiency of the intracellular killing mechanism of phagocytes
  2. Inability to generate oxygen free radicals
  3. Imparied killing of intracellular micro-organisms
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61
Q

What is the inability to clear organisms caused by in chronic granulomatous disease?

A

Excessive inflammation

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62
Q

What two features lead to excessive inflammation in chronic granulomatous disease?

A
  1. Failure to degrade chemoattractants and antigens

2. Persistent accumulation of neutrophils, activated macrophages and lymphocytes

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63
Q

In chronic granulomatous disease: failure of oxidative killing mechanisms - what does the inability to clear organisms ultimately result in?

A

Granuloma formation

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64
Q

Give 5 features of chronic granulomatous disease

A
  1. Recurrent deep bacterial infections
  2. Recurrent fungal infections
  3. Failure to thrive
  4. Lymphadenopathy and hepatosplenomegaly
  5. Granuloma formation
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65
Q

What 4 recurrent deep bacterial infections are especially prominent in chronic granulomatous disease?

A

Staphylococcus
Aspergillus
Pseudomonas cepacia
Mycobacteria, atypical mycobacteria

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66
Q

What investigation would you do for chronic granulomatous disease?

A

NBT (“nitroblue tetrazolium”) test

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67
Q

Give the three steps in the method for NBT testing in chronic granulomatous disease

A
  1. Feed patient neutrophils source of E.coli
  2. Add dye that is sensitive to H2O2
  3. If hydrogen peroxide is produced by neutrophils, due changes colour
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68
Q

Give two supportive treatment options for chronic granulomatous disease?

A

Prohpylactic antibiotics

Prophylactic antifungals

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69
Q

Give two definitive treatments for chronic granulomatous disease?

A

Stem cell transplantation

Gene therapy

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70
Q

What is special about these three intracellular organisms - Salmonella, Chlamydia, Rickettsia?

A

They hide from immune system by locating within cells

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71
Q

What is clever about the way mycobacteria species hide in the body?

A

They are intracellular organisms and hide within immune cells

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72
Q

What does an infection with mycobacteria (TB) activate?

A

IL-12 - gIFN network

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73
Q

What is the first step in IL-12 - gIFN network?

A

Infected macrophages stimulated to produce IL-12

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74
Q

IL-12 - gIFN network: after IL-12 has been produced by infected macrophages, what occurs?

A

IL-12 induces T cells to secrete gamma interferon (gIFN)

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75
Q

During IL-12 - g-IFN network - what occurs after T cells have secreted gIFN?

A

gIFN feeds back to macrophages and neutrophils

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76
Q

During IL-12 - gIFN network: what occurs after gIFN has fed back to macrophages and neutrophils?

A

It stimulates the production of TNF

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77
Q

During IL-12 - gIFN network: what occurs after the production of TNF?

A

TNF activates NADPH oxidase which stimulates oxidative pathways

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78
Q

Give 3 single gene defects in the IL-12 - gIFN axis?

A

gIFN receptor deficiency
IL-12 deficiency
IL-12 receptor deficiency

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79
Q

Name an important side effect of anti-TNF therapy?

A

Reactivation of latent tuberculosis

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80
Q

Name the two phagocyte recruitments

A
  1. Mobilisation from bone marrow

2. Migration to site of infection

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81
Q

How would you investigate the phagocyte recruitment: mobilisation from bone marrow?

A

Full blood count and differential

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82
Q

How would you investigate phagocyte recruitment: migration to site of infection?

A

Presence of pus, expression of neutrophil adhesion molecules

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83
Q

How would you investigate chemotaxis and endocytosis in phagocyte function?

A

Chemotactic assays

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84
Q

How would you investigate the formation of phagolysosomes in phagocyte function?

A

Phagocytosis assays

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85
Q

How would you investigate oxidative killing in phagocyte function?

A

NBT test of oxidative killing

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86
Q

What are is the neutrophil count, pus formation, leukocyte adhesion markers and nitroblue test of oxidative killing (NBT) like in congenital neutropaenia?

A

Neutrophil count - absent
Pus formation - No
Leukocyte adhesion markers - Normal
NBT - usually absent (because no neutrophils)

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87
Q

What are is the neutrophil count, pus formation, leukocyte adhesion markers and nitroblue test of oxidative killing (NBT) like in leukocyte adhesion defect?

A

Neutrophil count - Increased during infection
Pus formation - No
Leukocyte adhesion markers - Absent
NBT - Normal

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88
Q

What are is the neutrophil count, pus formation, leukocyte adhesion markers and nitroblue test of oxidative killing (NBT) like in chronic granulomatous disease?

A

Neutrophil count - normal
Pus formation - yes
Leukocyte adhesion markers - normal
NBT - abnormal

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89
Q

What two ways can phagocyte deficienceies be treated?

A

Aggressive management of infection

Definitive therapy

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90
Q

In aggressive management of phagocyte deficiencies, what two drugs are used in infection prohpylaxis?

A

Septrin

Itraconazole - antifungal

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91
Q

In the aggressive management of infection in phagocyte deficiencies, what other than infection prophylaxis, can be used for treatment?

A

Oral/intravenous antibiotics

Surgical draning of abscesses

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92
Q

Give two definitive therapies for treatment of phagocyte deficiencies?

A

Bone marrow transplantation

Specific treatment for Chronic granulomatous disease - gamma interferon therapy/gene therapy

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93
Q

What is severe congenital neutropaenia and cyclic neutropaenia caused by?

A

Failure of neutrophil differentiation

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94
Q

What does failure to express leukocyte adhesion markers give rise to?

A

Leukocyte adhesion deficiencies

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95
Q

What disease results from failure of oxidative killing?

A

Chronic granulomatous disease

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96
Q

What does a failure of cytokine production lead to?

A

gIFN and IL-12 deficiency

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97
Q

What type of response is the recognition by preformed, non-specific effectors via pattern recognition molecules. Recruitment and activation of phagocytes and complement?

A

Innate immune response

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98
Q

What do migrating dendritic cells initiate?

A

Adaptive immunity

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99
Q

What immunity is responsive to an unlimited number of molecules, has specificity and memory and its repertoire is not genetically encoded?

A

Acquired

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100
Q

What two organs beginning with ‘a’ are involved in the aquired immune response?

A
  1. Adenoid

2. Appendix

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101
Q

What three anatomical parts are involved in the aquired immune response, beginning with “t”?

A

Tonsil
Thymus
thoracic duct

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102
Q

Which two veins are involved in the squired immune response?

A

Subclavian vein (left and right)

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103
Q

Which three anatomical parts beginning with “L” are involved in the adaptive immune response?

A

Lymph nodes
Lymphatics
Large intestine

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104
Q

Is bone marrow part of the anatomy of the acquired immune response?

A

Yes

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105
Q

Which anatomical part beginning with “p”, “k”, “h” and “s” are involved in the acquired immune response?

A

Peyer’s patch in the small intestine
Kidneys
Heart
Spleen

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106
Q

Where do T lymphocytes arise from?

A

Haematopoetic stem cells in bone marrow

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107
Q

Where are immature T cells exported to from the bone marrow?

A

To the thymus for proliferation and maturation

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108
Q

What percentage of T cells survive selection in the thymus?

A

10%

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109
Q

Once mature T lymphocytes have entered the circulation, where do they reside?

A

In lymph nodes and secondary lymphoid follicles

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110
Q

What cells provide defence against intracellular pathogens and viruses and immunoregulation?

A

T lymphocytes

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111
Q

What are the two main groups of T cells?

A

CD4 and CD8 (each with different function and different expression of cell surface proteins

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112
Q

Which type of T cells have immunoregulatory functions and provide costimulatory signals?

A

CD4+

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113
Q

What two roles do the costimulatory signals from CD4 T cells have?

A
  1. Necessary for activation of CD8 T lymphocytes nad naive B cells
  2. Also influence phagocyte function
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114
Q

Other than providing costimulatory signals, what do CD4 T lymphocytes produce and regulate?

A

Produce cytokines

Regulate other lymphocytes and phagocytes

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115
Q

Which type of T cell recognises peptides presented on HLA class II molecules?

A

CD4+ T lymphocytes

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116
Q

Which thee peptides in association with HLA class I do CD8+ cells recognise?

A

HLA-A
HLA-B
HLA-C

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117
Q

In what three ways do cytotoxic T cells kill other cells directly?

A

Production of pore-forming molecules: perforin
Triggering apoptosis of the target
Secrete cytokines e.g. IFNgamma

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118
Q

Name a production of pore-forming molecules used by T cells to kill cells?

A

Perforin

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119
Q

Where do B cells arise from?

A

Haemopoetic stem cells in bone marrow

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120
Q

Where are mature B lymphocytes mainly found?

A

In bone marrow, lymphoid tissue, spleen

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121
Q

What two functions fo B cells have?

A

Antibody production

Antigen presentation

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122
Q

What are the 4 changes of B cell in development in the bone marrow?

A
  1. Stem cells
  2. Lymphoid progenitors
  3. Pro B cells
  4. Pre B cells
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123
Q

What do Pro B cells express?

A

IgM

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124
Q

What do pre B cells express?

A

IgM and IgD

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125
Q

What four other cells can IgM B cells go on to mature into?

A

IgM plasma cells
IgA
IgE
IgG

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126
Q

Where do antigen encounters with B cells occur?

A

Within lymph nodes

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127
Q

What do B cells within the lymph nodes need to rapidly proliferate?

A

Appropriate signals from T lymphocytes

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128
Q

What is meant by B cells undergoing highly complex genetic rearrangements?

A

Generate B cells that express receptors of greater affinity than the original

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129
Q

What two cells can T cell stimulated B cells further differentiate into?

A

Long-lived memory cells

Plasma cells which produce antibody

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130
Q

Give four functions of antibodies

A
  1. Identification of pathogens
  2. Rcruitment of other componenets of immune response to remove pathogens
  3. Neutrilisation of toxins
  4. Particularly important in defence agaisnt bacteria of all kinds
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131
Q

What three componenets of immune response can antibodies recruit?

A

Complement
Phagocytes
NK cells

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132
Q

What is a defect of haemopoetic stem cells called, that makes the adaptive immune response go wrong?

A

Reticular dysgenesis

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133
Q

What leads to the failure of production of Neutrophils, Lymphocytes, Monocytes/macrophages, platelets and is fatal unless corrected with bone marrow transplantation?

A

Recticular dysgenesis

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134
Q

What disease is associated with failure of production of lymphocytes?

A

Severe combined immunodeficiency

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135
Q

How long before a patient with severe combined immunedeficiency becomes unwell?

A

3 months of age

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136
Q

Do you get persistent diarrhoea with severe combined immunodeficiency?

A

Yes

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137
Q

What significant clinical feature is present in severe combined immunodeficiency?

A

Graft versus host disease (skin disease)

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138
Q

What does colonisation of infants “empty” bone marrow by maternal lymphocytes cause?

A

Graft versus host disease

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139
Q

Which antibody can cross the placenta?

A

IgG

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140
Q

Between 0 months and 6 months, where does the neonate get IgG from?

A

Colostrum and breast milk

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141
Q

What is SCID?

A

Severe Combined Immuno Deficiency

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142
Q

What protects the SCID neonate in the first 3 months of life?

A

Maternal IgG

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143
Q

What is the term for normal babies getting infections at 3-4 months if their immune systems are slow to mature?

A

Transient hypogammaglobulinaemia of infancy

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144
Q

Name 4 pathways which can cause severe combined immunodeficiency

A
  1. Deficiency of cytokine receptors
  2. Deficiency of signalling molecules
  3. Metabolic defects
  4. Defective receptor arrangements
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145
Q

What is the commonest form of Severe combined immunodeficiency?

A

X-linked SCID

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146
Q

What is the mutation in X-linked SCID?

A

Mutation of componenet of IL-2 receptor

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147
Q

What receptor is shared by many other cytokine receptors?

A

IL-2 receptor

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148
Q

Which mutation in X-linked SCID results in inability to respond to cytokines and what two things does this lead to?

A

Mutation of componenet of IL-2- receptor

Failure of T cell and NK cell development
Production of immature B cells

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149
Q

What can be said about the T cell number, B cell number and development of lymphoid tissue and thymus in X-linked SCID?

A

T cells - very low or absent
B cells - normal or increased
Poorly developed lymphoid tissue and thymus

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150
Q

What are 3 methods of prophylactic treatment for SCID?

A
  1. Prophylactic antibiotics and antifungals
  2. Aggressive treatment of exisiting infections
  3. Antibody replacement - IV immunogloblin
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151
Q

Give two definitive treatments for SCID involving stem cells

A
  1. Stem cell transplant from HLA identical sibling if possible
  2. Stem cell transplant from other sibling or parent, or from matched unrelated donor
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152
Q

In gene therapy for SCID: can Stem cells be treated ex vivo to express the missing component?

A

Yes

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153
Q

What is DiGeorge syndrome?

A

Developmental defect of 3rd/4th pharyngeal pouch

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154
Q

What chromosome is deleted in DiGeorge syndrome?

A

22q11

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155
Q

What is CATCH-22 for DiGeorge syndrome?

A 
C - Cardiac defects
A - Abnormal facial features
T - Thymus underdevelopment
C - Cleft palate
H - Hypocalcemia
22 - Deletion of several genes in chromosome 22
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156
Q

What disease does a funny looking kid with: low set ears, abnormally folded ears, high forehead, cleft palate, small mouth and jaw, hypocalacemia, oesophageal atresia, T cell lymphopenia and complex congenital heart disease have?

A

DiGeorge Syndome

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157
Q

What does failure of thymic development in DiGeorge syndrome result in?

A

T cell immunodeficiency (nowhere for T cells to mature)

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158
Q

In DiGeorge syndrome, what comes before Hypocalcaemia?

A

Hypoparathyroidism

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159
Q

Which two developmental disorders can DiGeorge sybndrome cause?

A

Obsessive compulsive disorder

Schizophrenia

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160
Q

Which gene is responsible for embryonic development of the pharyngeal pouch?

A

TBX1

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161
Q

Name three types of infections someone with DiGeorge will get?

A

Recurrent viral infections
Recurrnet bacterial infections
Frequent fungalo infecitons

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162
Q

In a laboratory investigation of DiGeorge syndrome - what can be said about: T cell number and activation response, B cell numbers and antibody responses and NK cell numbers?

A

T cells - absent or decreased, defective T cell activation response
B cells - normal or increased, low IgG, IgA, IgE, poor antibody responses to specific pathogens
Normal NK numbers

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163
Q

With DiGeorge syndrome, what happens to T cell function through ageing?

A

Improves

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164
Q

What 4 things can be done as management for DiGeorge syndrome?

A

Correct metabolic/cardiac abnormalities
Prophylactic antibiotics
Early and aggressive treatment of infection
Some patients require immunoglobulin replacement

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165
Q

What three features can disorders of T cell effector function affect?

A

Cytokine production
Cytotoxicity
T-B cell communication

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166
Q

What does deficiency of any componenet of cytokine production (T cell effector function) result in?

A

Susceptibility to infection such as TB, BCG, Aspergillus

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167
Q

What disease is failure of lymphocyte precursors?

A

SCID

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168
Q

What disease involves failure of thymic development?

A

DiGeorge syndrome

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169
Q

What disease involves failure of expression of HLA molecules?

A

Bare lymphocyte syndromes

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170
Q

What two deficiencies are associated with failure of signalling, cytokine production and effector functions?

A

gIFN deficiency and IL-12 deficiency

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171
Q

What disease is associated with failure of normal apoptosis?

A

Autoimmune lymphoproliferative syndromes

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172
Q

What are recurrent infections (viral, fungal, bacterial, intracellular), opportunistic infections, malignancies young and autoimmune disease all clinical features of?

A

T cell deficiencies

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173
Q

What are the 3 first line investigations for T cell deficiencies?

A
  1. Total WCC and differential
  2. Serum immunoglobulins and protein electrophoresis - surrogate marker of functional T cells
  3. Quantification of lymphocyte subpopulations
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174
Q

What are the 3 second line investigations for T cell deficiencies?

A
  1. Functional tests of t cell activation and proliferation - may be useful if signalling or activation defects are suspected
  2. Additional tests of lymphocyte lineage
  3. An HIV test is essential
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175
Q

Are lymphocyte counts higher in children or adults?

A

Children

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176
Q

If a patient is getting recurrent upper and lower respiratory tract infections, recurrent GI infections, often common organisms what is going on?

A

Antibody deficiency

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177
Q

Name 2 antibody mediated autoimmune diseases?

A
  1. Idiopathic thrombocytopaenia

2. Autoimmune haemolytic anaemia

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178
Q

What occurs when there is failure to produce mature B cells (Pro B cells cannot make Pre B cells)?

A

Bruton’s X-linked hypogammaglobulinaemia

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179
Q

What disease is there no circulating B cells, no plasma cells and no circulating antibody after the first 6 months?

A

Bruton’s X-linked hypogammaglobulinaemia

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180
Q

What is the prevelance for selective IgA deficiency?

A

1:600

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181
Q

What disease has low IgG, IgA and IgE, recurrent bacterial infections, cause and mechanism is unknown and often associated with autoimmune disease?

A

Common variable immuno deficiency

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182
Q

In common variable immune deficiency - what three immunoglobulins are low?

A

IgG, IgA, IgE

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183
Q

What are 3 clinical features of common variable immune deficiency?

A
  1. Recurrent bacterial infections
  2. AAutoimmune disease
  3. Granulomatous disease
184
Q

What disease is failure of B cell maturation?

A

X-linked agammaglobulinaemia

185
Q

What disease is failure of T cell costimulation?

A

X-linked hyper IgM syndrome

186
Q

What disease is failure of IgA production?

A

Selective IgA deficiency

187
Q

Which two diseases involve failure of production of IgG antibodies?

A

Common variable immuen deficiency

Selective antibody deficiency

188
Q

What are the 3 first line investigations for B cell deficiencies?

A
  1. Total white cell count and differential
  2. Serum immunoglobulins
  3. Serum and urine protein electrophoresis
189
Q

What are 2 second line investigations for B cell deficiencies?

A

Quantification of B and T lymphocytes

Specific antibody responses to known pathogens

190
Q

What is one method of investigating specific antibody responses to known pathogens in B cell deficiencies?

A

Measure IgG antibodies agaisnt tetanus, Haemophilus influenzae B and S. pneumoniae

191
Q

What are the three management methods for B cell deficiencies?

A
  1. Aggressive treatment of infection
  2. Immunoglobulin replacement
  3. Stem cell transplantation in some situations
192
Q

Name 3 lymphoproliferative disease?

A

Chronic lymphocytic leukaemia
Myeloma
Non-Hodgkins lymphoma

193
Q

What two diseases can lead to protein loss?

A

Protein losing enteropathy

Nephrotic syndrome

194
Q

What investigation strategy would you use for hypogammaglobulinaemia (4 steps)?

A
  1. Lymphocyte subsets
  2. Repeat immunoglobulins, serum and urine electrophoresis and total proteins
  3. CT
  4. Check sepcific antibodies and consider test immunisation
195
Q

In selective IgA deficiency: what are the serum immunoglobulins and lymphocyte subpopulations like?

A 
IgM - no
IgG - often raised
IgA /
IgE - No
B cells - no
T cells - no
196
Q

What are the serum immunoglobulins and lymphocyte subpopulations for Common variable immune deficiency?

A 
IgM - decreased
IgG - decreased
IgA - decreased
IgE - decreased
B cell - variable
T cell - variable
197
Q

What other investigation would you do for common variable immune deficiency?

A

Failrue to produce specific antibodies after test immunisation

198
Q

What would the serum immunoglobulins and lymphocyte subpopulations be like for specific antibody deficiency?

A

All N, IgA N decreased

199
Q

What are immune responses that result in bystander damage to the self?

A

Hypersensitivity reactions

200
Q

What are the 4 classifications of hypersensitivity reactions?

A

Type I - Immediate hypersensitivity
Type II - Direct cell killing
Type III - Immune complex mediated
Type IV - Delayed type hypersensitivity

201
Q

What type of hypersensitivity do allergic diseases come under?

A

Immediate (Type I)

202
Q

What is the definition of an allergy?

A

IgE-mediated antibody response to external antigen

203
Q

Name 3 non-IgE mediated reactions

A
  1. Coeliac disease
  2. Eosinophilic gastroenteritis
  3. Protein induced enteropathy
204
Q

Why is the prevalence of allergy increasing?

A

Hygiene hypothesis

205
Q

How quickly do type I allergic diseases occur after exposure to allergen?

A

minutes - 1-2 hours

206
Q

What can the threshold for type I allergic disease be influenced by?

A

Cofactors such as exercise, alcohol and infection

207
Q

What are asthma, urticaria, angioedema, allergic rhinitis, allergic conjunctivitis, diarrhoea and vomiting and anaphylaxis all a type of?

A

Type I (immediate hypersensitivity) allergic disease

208
Q

Name 6 allergens

A
  1. House dust mite
  2. Pollen and animal dander
  3. Foods
  4. Drugs
  5. Latex
  6. Bee and wasp venom
209
Q

What are many allergens?

A

Soluble proteins that function as enzymes

210
Q

Name 3 types of cells involved in allergic disease

A
  1. B cells
  2. T cells
  3. Mast cells
211
Q

What type of cells in allergic disease recognise the antigen and produce antigen-specific IgE antibodies?

A

B lymphocytes

212
Q

What cells in allergic disease provide help for B lymphocytes to make IgE antibody?

A

T lymphocytes

213
Q

What are mast cells

A

Inflammatory cells that release vasoactive substances

214
Q

Where are mast cells found?

A

Resident in tissues, especially at interface with external environment

215
Q

What two classes of vasoactive substances do mast cells produce?

A

Preformed

Synthesised on demand

216
Q

Name 3 preformed vasoactive substances produced from mast cells?

A

Histamine
Tryptase
Heparin

217
Q

Name 3 synthesised on demand vasoactive substances produced by mast cells

A
  1. Leukotrienes
  2. Prostaglandins
  3. Cytokines including IL-4 and TNF
218
Q

What 4 steps do mast cells do in the orchestration of the inflammatory cascade?

A

Increase blood flow
contraction of smooth muscle
increase vascular permeability
increase secretions at mucosal surface

219
Q

What are mast cells important in the defense against?

A

Parasites and wound healing

220
Q

What do mast cells express on their surface, that are relevant in allergic reactions?

A

Express receptors for Fc region of IgE antibody on their surface

221
Q

On encounter with an allergen, what do B cells produce?

A

Antigen-specific IgE antibody

222
Q

What is the antigen specific region of an antibody called, and where is it located?

A

Fab

At top of antibody

223
Q

Where is the Fc region of an antibody and what region is it?

A

Constant region

Bottom of antibody

224
Q

What occurs after re-encounter with an antigen?

A

Allergen binds to IgE-coated mast cells are disrupts cell membrane. The release of vasoactive mediators - histamine, tryptase, also increased cytokines nad leukotriene transcription.

225
Q

Once the allergen is initially cleared, what do residual IgE antibodies do?

A

Bind to circulating mast cells via Fc receptors

226
Q

Clinical features of allergic disease: within minutes of release of vasoactive mediators, clinical manifestations occur - name 5

A
  1. Urticaria
  2. Angioedema
  3. Asthma
  4. Allergic rhinitis and conjunctivitis
  5. Anaphylaxis
227
Q

What two types of asthma can you get?

A

Extrinsic and intrinsic

228
Q

What type of asthma is a response to external allergen and IgE mediated?

A

Extrinsic asthma

229
Q

What type of asthma is intrinisc asthma?

A

Non-allergic asthma and not IgE mediated

230
Q

In extrinsic asthma - what occurs after re-encounter with antigen?

A

Allergen binds to IgE coated mast cells
Disrupts cell membrane
Causes release of vasoactive mediators from primary granules (histamine, tryptase)
Increases production of other cytokines and leukotrienes

231
Q

What occurs in response to the release of histamine and other inflammatory mediators to muscle spasm, mucosal inflammation and inflammatory cell infiltrate?

A

Causes bronchoconstriction
Causes mucosal oedema and increases secretions
Infiltration of lymphocytes and eosinophils into bronchioles

232
Q

What clinical manifestation does bronchoconstriction give?

233
Q

What kind of clinical manifestation does mucosal oedema and increased secretions give?

A

Sputum production

234
Q

What kind of clinical manifestation does infiltration of lymphocytes and eosinophils into bronchioles cause?

A

Sputum often yellow

235
Q

Give another name for urticaria

A

Hives, wheals, nettle rash, blisters

236
Q

What is angioedema?

A

Self-limited, localised swelling of subcutaneous tissues or mucous membranes

237
Q

What changes can occur to breathing during anaphylaxis?

A

Wheeze
Bronchoconstriction
Laryngeal obstruction - stridor

238
Q

What can happen to the lips and mucous membranes during anaphylaxis?

A

Angioedema

239
Q

What 3 circulatory changes can occur during anaphylaxis?

A
  1. Hypotension
  2. Cardiac arrhythmias
  3. MI
240
Q

What can happen to the palsm and soles of feet during anaphylaxis?

241
Q

What 3 GI changes can occur during anaphylaxis?

A

Vomiting
Diarrhoea
Abdominal pain

242
Q

Give 4 non-allergic causes of mast cell degranulation?

A

Drugs
Thyroid disease
Idiopathic
Physical urticaria

243
Q

What is aspirin induced asthma characterised by?

A

Wheeze 0.5-3 hours after ingestion

244
Q

What two other NSAIDS can cause induced asthma other than aspirin?

A

Diclofenac

Ibuprofen

245
Q

What affects 2-3% of asthmatics?

A

Samter’s triad

246
Q

What is Samter’s triad?

A

Asthma
Nasal polyps
Salicylate sensitivity

247
Q

Give 3 elective investigations for allergiies?

A

Skin prick tests
Quantitive specific IgE to putative allergen
Challenge test - supervised exposure

248
Q

What investigation can be done to diagnose allergic reactions during an acute anaphylactic episode?

A

Evidence of mast cell degranulation - serum mast cell trpytase levels

249
Q

What is the gold standard for supporting diagnosis of allergy?

A

Skin prick test

250
Q

What is a postiive reaction in a skin prick test?

A

Local wheal and flare response

251
Q

Do corticosteroids influence skin prick tests?

252
Q

Do antihistamines influence skin prick tests?

A

Yes - discontinued 48 hours before testing

253
Q

What are RAST tests?

A

Specific IgE tests that measure amount of IgE in serum directed against specific allergen

254
Q

What are the most common causes of an elevated IgE in industrial societies?

A

Allergic disorders = high total IgE

255
Q

Can vasculitis cause elevated IgE?

256
Q

Is total IgE a useful routine test in the diagnosis/investigation of allergic disease?

257
Q

What does widespread degranulation of mast cells during anaphylaxis result in?

A

Increase in serum tryptase

258
Q

Management of IgE mediated allergic disorders: What is used to block mast cell activation?

A

Mast cell stabilisers - sodium cromoglycate

259
Q

How are mast cell stabilisers like sodium cromoglycate administered?

A

Topical spray when allergen exposure is predictable

260
Q

Management of IgE mediated allergic disorders: What drugs can be given to prevent the affect of mast cell activation?

A

Anti-histamines

Leukotriene receptor antagonists

261
Q

What are the mainstay of treatment of allergic disease?

A

Anti-histamines

262
Q

How do leukotriene receptor anatonists work and give an example?

A

Blocks effects of leukotrienes which are synthesised by mast cells after activation

Montelukast

263
Q

What do corticosteroids inhibit the formation of?

A

Many different inflammatory mediators

264
Q

Give three inflamamtory mediators that corticosteroids inhibit the formation of?

A

Platelet activating factor
Prostaglandins
Cytokines

265
Q

What drugs are often used in aspirin-sensitive individuals for asthma?

A

Leukotriene receptor antagonists

266
Q

What drug would you use in exercise-induced asthma?

A

Mast cell stabiliser - sodium cromoglycate

267
Q

What do you add on next as well as use of an inhalted short acting beta-2-adrenoceprot agonist?

A

Low dow inhaled corticosteroids

268
Q

What is the primary management in anaphylaxis?

A

Self-injectable adrenalin

269
Q

What does adrenaline act on and what does it cause?

A

B2 adrenergic receptors to constrict arterial smooth muscle

270
Q

What two effects does the constriction of arterial smooth muscle by adrenaline cause?

A
  1. Increases blood pressure thereby limiting vascular leakage
  2. Dilates bronchial smooth muscle, thereby decreasing airflow obstruction
271
Q

What is immunotherapy in terms of anaphylaxis management?

A

Controlled exposure to increasing amounts of allergen

272
Q

What is hypersensitivity II?

A

Direct cell killing

273
Q

What is the key feature of direct cell killing?

A

Antibody to cell surface antigens

274
Q

In the pathophysiology of direct cell killing - what occurs once the antibody has binded to cell-surface antigen?

A

Activation of complement - cell lysis and opsonisation

Antibody mediated phagocytosis

275
Q

What immunoglobulins do B cells produce directed against cell membrane protein?

A

IgM or IgG

276
Q

What do classical C1, C2, C4, lectin MBL pathway and alternative pathway all lead to?

277
Q

What is formed at the end of the complement cascade?

A

Membrane attack complex

278
Q

What are the 4 effects of complement activation?

A
  1. Chemotaxis
  2. Solubilsation
  3. Direct killing
  4. Opsonisation
279
Q

What is chemotaxis?

A

Stimulation of migration of macrophages and neutrophils to site of inflammation

280
Q

What is solubilisation?

A

Solubilisation of immune complexes

281
Q

What does direct killing do?

A

Direct killing of encapslated bacteria

282
Q

What does opsonisation do?

A

Enhance phagocytosis by macrophages and neutrophils

283
Q

What punches holes in bacterial membranes?

A

Membrane attack complex

284
Q

What increases vascular permeability and increases cell trafficking to site of inflammation?

A

Chemotaxis

285
Q

What beginning with “c” can increase vascular permeability?

A

Complement

286
Q

What are anaphylotoxins?

A

Fragments of complement proteins released after activation increase permeability of blood vessels

287
Q

What fragments act to solublise immune complexes?

A

Complement

288
Q

What is triggered by immune complexes?

A

Complement activation

289
Q

How could fragments of complement switch off the process of complement activation?

A

They can dissolve the immune complexes which triggered them, switching off the process of complement activation

290
Q

Which effect of complement activation causes regulation of pathway through negative feedback?

A

Solubilsation

291
Q

In type II hypersensitivity reaction - what does the binding of IgG or IgM antibody to cell surface antigen result in?

A

Complement activation

292
Q

Give a clinical example of type II hypersensitivity

A

Blood transfusion reactions (ABO reactions)

293
Q

In an ABO reaction - what are the 5 steps?

A
  1. Target is donor red cells
  2. Anti-blood group antibodies bind to surface of circulating donor erythrocytes
  3. Form antigen-antibody complexes
  4. Activates complement
  5. Stimulates phagocytosis
294
Q

What is an overwhelming systemic inflammatory response including pyrexia and rigors, tachycardia/tachypnoea, hypotension/dizziness, headaches/chest or lumbar pain all part of?

A

Immediate haemolytic transfusion reaction

295
Q

Give three reactions that are type II hypersensitivity blood cell reactions

A
  1. Transfusion reactions
  2. Autoimmiune haemolytic anaemia
  3. Idiopathic thrombocytopaenic purpura
296
Q

Name a type II hypersensitivity reaction to do with the kidney

A

Goodpastures sndrome (antibodies to glomerular basement membrane)

297
Q

Give 2 diseases that are hypersensitivity type II and associated with the nervous system

A
  1. Myasthenia gravis (antibodies to acetyl choline receptor)

2. Guillan Barre syndrome (antibodies to peripheral nerve glycoprotein)

298
Q

Give a type II hypersensitivity reaction related to the endocrine system

A

Graves’ disease (antibodies to TSH receptor)

299
Q

Give a type II hypersensitivity reaction to do with the skin

A

Pemphigus vulgaris (antibodies to epithelial cell cement)

300
Q

How could you prove that the disease is mediated by autoantibodies?

A

Demonstrate that serum causes disease when transferred into another host

301
Q

Nmae 3 diseases that may be transferred to the neonate

A
  1. Myasthenia gravis
  2. Idiopathic thrombocytopaenic purpura
  3. Rhesus disease
302
Q

What are the two main managements for type II hypersensitivity?

A

Plasmapheresis

Immunosuppresion

303
Q

What is the aim of plasmopheresis?

A

Removal of pathogenic antibody

304
Q

What management method involves: cellular constituents of blood replaced, plasma replaced by plasma from someone else (FFP) or pooled immunoglobulin approx 50% plasma removed each time?

A

Plasmapheresis

305
Q

What does rebound antibody production limit the efficacy of?

A

Plasmapheresis

306
Q

What type of hypersensitivity is type III?

A

Immune complex mediated

307
Q

Where can immune complexes deposit?

A

In small vessels

308
Q

Name a type III hypersensitivitry disease

A

Farmer’s lung

309
Q

What causes Farmer’s lung?

A

Inhaled fungal particles from hay deposited in lung

310
Q

In Farmer’s lung - what happens after antibody formation has been stimulated by inhaled fungal particles?

A

Antibodies form immune complexes with antigen

Results in complement activation, inflammation and recruitemnt of other cells

311
Q

What clinical features does Farmer’s lung cause?

A

Wheezing and malaise

312
Q

Name two causes of hypersensitivity pneumonitis?

A

Farmer’s lung - mouldy hay, aspergillus fumigatus

Bird fancier’s lung - avian excreta, avian serum proteins

313
Q

What does immune complexes deposited in the walls of alveoli and bronchioles cause?

A

Acute hypersensitivity pneumonitis

314
Q

What occurs within alveoli during acute hypersensitivity pneumonitis?

A

Cell accumulation and inflammation

315
Q

What type of response is acute hypersensitivity pneumonitis mediated by?

A

Type III hypersensitivity

316
Q

What does acute hypersensitiity pneumonitis cause after 4-8 hours after exposure to antigen, and what is it associated with?

A

Wheezing and malaise

Associated with dry cough, pyrexia and breathlessness

317
Q

What clinical feature does inflammation of terminal bronchioles and alveoli caused by activated phagocytes and copmplement cause?

318
Q

What clinical feature does alveolitis, caused by activated phagfocytes and complement resulting in decreased efficiency of gas trasfer cause?

A

Breathlessness

319
Q

What clinical feature does systemic manifestation of inflammatory response cause?

A

Malaise and pyrexia

320
Q

What type of reaction is systemic lupus erythematosus (SLE)?

A

Type III hypersensitivity

321
Q

What are the 4 stages in systemic lupus erythematosus (SLE)?

A

Antibodies produced against contents of cell nuclei
Form immune complexes
Depositied in small vessels in skin, joints, kidneys
Result in complement activation…iflammation and recruitment of other cells

322
Q

What is small vessel vasculitis caused by?

A

Immune complex depososition

323
Q

What does deposition of IgG immune complexes in glomeruli cause?

A

Renal dysfunction

324
Q

What does immune complex deposition in joints cause?

A

Arthralgia

325
Q

What does immune complex deposition in skin cause?

A

Vasculitic purpura

326
Q

What is used to diagnose (SLE)?

A

Anti-DNA binding antibodies

327
Q

What is used to diagnose Farmer’s lung?

A

Antibodies to aspergillus

328
Q

What two factors can you decrease to manage type III hypersensitivity reactions?

A

Decrease inflammation - corticosteroids

Decrease production of antibody - immunosuppression

329
Q

Can restrictive lung diseases such as sarcoid and pulmonary fibrosis be treated with corticosteroids?

330
Q

Can sleep apnoea be treated with corticosteroids?

331
Q

Can infective lung diseases be treated with corticosteroids?

332
Q

Can uncomplicated infection be treated with corticosteroids?

333
Q

What is hypersensitivity type IV?

A

Delayed type hypersensitivity

334
Q

What cells mediate type IV hypersensitivity?

335
Q

What hypersensitivity does this describe: Initial sensitisation to antigen generates “primed” T cells, subsequent exposure leads to activation of “primed” T cells, recruitment of macrophages, lymphocytes and neutrophils and release of proteolytic enzymes?

A

Delayed type hypersensitivity

336
Q

Give an example of type IV hypersensitivity reaction?

A

Nickel hypersensitivity

337
Q

What is a collection of activated macrophages and lymphocytes called?

338
Q

What hypersensitivity reaction does a granuloma form in?

339
Q

What are the three steps in type IV hypersensitivity reaction?

A
  1. Infiltration of activated T cells
  2. Recruitment of macrophages - phagocytosis, production of inflamamtory cytokines
  3. Granuloma formation
340
Q

During a type IV hypersensitivity reaction: what 4 things do CD4 T cells do in the initial phase?

A
  1. production of cytokines
  2. Recruitment and activation of macrophages
  3. Help for CD8 lymphocytes
  4. Granuloma formation
341
Q

Give 3 autoimmune diseases associated with delayed type hypersensitivity

A
  1. Type 1 diabetes
  2. Psoriasis
  3. Rheumatoid arthritis
342
Q

What type of reactions are: nickel hypersensitivity, TB, leprosy, sarcoidosis and cellular rejection of solid organ transplant?

A

Non autoimmune delayed type hypersensitivity reactions

343
Q

What is sarcoidosis?

A

Multisystem granulomatous disease, characterised by presence of granulomas

344
Q

What is a granuloma?

A

An organised collection of activated macrophages and lymphocytes

345
Q

What are the 4 steps of sarcoidosis pathophysiology?

A
  1. Inhalation of unknown antigen
  2. Stimulates alveolar macrophages and CD4 and CD8 T cells, and B cells within lung parenchyma
  3. Failure to clear the antigen results in persistent stimulation and granuloma formation
  4. Persistent immune activation leads to tissue damage and fibrosis
346
Q

What can be given for the management of acute onset of sarcoidosis?

347
Q

What do corticoisteroids block in the management of sarcoid?

A

T cell activation

Macrophage activation

348
Q

Give 5 diseases characterised by type IV hypersensitivity and granuloma formation

A
  1. Sarcoidosis
  2. TB
  3. Leprosy
  4. Berylliosis, silicosis and other dust diseases
  5. Chronic stage of hypersensitivity pneumonitis
349
Q

What is vaccination?

A

Deliberate exposure to an antigen

350
Q

Why are vaccinations done?

A

In order to induce immunologically mediated resistance to disease

351
Q

How do vaccinations work?

A

Through the induction of memory

352
Q

What long-lived cells are generated during primary humoral immune responses?

A

Memory B cells

353
Q

When do memory B cells undergo clonal expansion, differentiation into plasma cells and antibody production?

A

When they are rapidly reactivated to a second encounter with that specific antigen

354
Q

During the secondary antibody response - what antibody blocks bacterial attachement to mucous membranes?

A

Preformed IgA

355
Q

What is the name for: individual may clear toxin through anti-toxin antibodies, but remain a carrier of microorganism?

A

Diphtheria

356
Q

What type of cells does vaccination stimulate?

A

Rare naive T cells

357
Q

What are the two fates of effector T cells, induced by vaccination?

A
  1. Mostly die by apoptosis in the absence of persisting antigen
  2. Smaller number become memory cells and are maintained at low frequency
358
Q

What are primed CD4 T cells able to do immediatly?

A

Produce cytokines

359
Q

What are primed CD8 T cells able to do?

A

Kill immediatly without requirement of immunological help

360
Q

What are two broad classes of vaccination?

A

Active and passive

361
Q

What two classes can active vaccination be split into?

A

Live attenuated and inactive

362
Q

Name three classes inactive vaccinations can be put into?

A

Killed
Subunit
Toxoid

363
Q

What is the term for the process through which an individual develops immunity/memory to a disease, including natural infection?

A

Immunisation

364
Q

What is the term for the delibrate administration of antigenic material to produce immunity to a disease?

A

Vaccination

365
Q

What is the term of immunity: protection is produced by the persons own immune system, can be stimulated by vaccine or naturally acquired infection, usually permanent?

A

Active immunity

366
Q

What type of immunity: protection is transferred from another person or animal, temporary protection that wanes with time?

A

Passive immunity

367
Q

How does an active vaccination stimulate immune responses to antigens?

A

Through same pathways as natural infection

368
Q

What is another name for the novel H1N1 flu virus?

369
Q

Give two methods of exposing an individual to the infectious organism?

A

Variolation

Swine flu parties

370
Q

Give an example of exposure to a similar but less virulent pathogen?

A

Cowpox protects from smallpox

371
Q

What is another term for inactivated vaccines?

A

Killed vaccines

372
Q

What vaccines: cannot replicate, not as effective as live vaccines, immune response primarily antibody based, antibody titer may diminish with time, require multiple doses to stimulate immune response?

A

Inactivated vaccines

373
Q

What are three ways to make an inactivated vaccine?

A
  1. Chemical fixatives e.g. formalin
  2. Heat denaturation
  3. Irradiation
374
Q

Give two problems with inactivated vaccines

A
  1. Under-inactivation - leaves viable pathogens or toxins within organism
  2. Over-inactivation - loss of tertiary structure and conformational antibody binding site
375
Q

Do inactivated vaccines need refrigeration?

376
Q

What vaccines require multiple injections and booster immunisation?

A

Inactivated vaccines

377
Q

What are adjuvants?

A

Mixture of inflammatory substances required to stimulate immune responses to coadministered peptides, proteins or carbohydrates

378
Q

Name 3 adjuvants

A
  1. Aluminium hydroxide
    Toxins e.g. tetanus toxoid
    Bordetella pertussis
379
Q

Why do adjuvants work?

A

Create inflammatory environment

380
Q

In what two ways do adjuvants work by?

A
  1. Bind to macrophages and signal the unequivocal presence of a microbial invader
  2. Activate the innate immune system to stimulate development of antibody and T cell responses
381
Q

Give two problems of adjuvants

A

Toxic

Alter immune response

382
Q

What are these three features a probelm of? Immune response is generated to vaccine:protein conjugates rather than vaccine itself, CD4 T cells may recognise the carrier only?

383
Q

What are inactivated vaccines called when they used the whole organism?

A

Whole cell vaccines

384
Q

Give some examples of whole cell inactivated vaccines

A 
Polio (Salk)
Hepatitis A
Rabies
Cholera
Plague
385
Q

What is the name for inactivated vaccines where only part of the organism is used?

A

Fractional vaccines

386
Q

What are the three subgroups of fractional inactivated vaccines?

A

Subunit vaccines
Toxoid
Pure polysaccharide vaccines

387
Q

Give some examples of subunit vaccines?

A 
Hepatitis B
Influenza
Acellular pertussis
HPV
Anthrax
388
Q

Give two examples of toxoid fractional inactivated vaccines?

A

Diphtheria

Tetanus

389
Q

What do polysaccharides of outer capsules of some bacteria determine?

A

Their pathogenicity and antigenicity

390
Q

Give three examples of polysaccharide only vaccines?

A

Pneumovax (pneumococcus)
Meningovax (MenC)
Salmonella Typhi (Vi)

391
Q

Give two examples of polysaccharide conjugated to toxin, polysaccharide vaccines?

A

Haemophilus infleunze type B

Prevenar (pneumococcus)

392
Q

What do live attenuated vaccines contain?

A

Weakened form of the ‘wild’ virus or bacterium

393
Q

What must the organism in a live attenuated vaccine do to be effective?

394
Q

What type of vaccines are these an example of: measles, mumps, rubella, chickenpox, yellow fever, rotavirus, smallpox and polio (Sabin)?

A

Live attenuated vaccines

395
Q

Name two bacterial vaccines that are live attenuated?

A

BCG

Oral typhoid

396
Q

Do live attenuated vaccines need boosting?

397
Q

Give three disadvantages of live attenuated vaccines?

A

Immune response can be interfered with by circulating antibody
Safety - immunocompromised hosts
Fragile - cold chain

398
Q

Which is inactivated and which is live attenuated: Salk or Sabin?

A

Salk - inactivated

Sabin - live attenuated

399
Q

Give an example of passive immunity?

A

Maternal antibody

400
Q

Name 4 sources of therapeutic passive immunisation

A
  1. Pooled normal human immunoglobulin
  2. Hyperimmune globulin
  3. Heterologous hyperimmune serum
  4. Monoclonal antibody agaisnt specific pathogen
401
Q

When is hyperimmune globulin adminsitered?

A

After specific exposure

402
Q

What is the name of a monoclonal antibody produced against a single determinant of the Respiratory Syncitial Virus (RSV)?

A

Palivizumab

403
Q

How is palivizumab administered?

A

Intramuscular

404
Q

What are the two types of cancer vaccines?

A

Preventative vaccines and therpeutic vaccines

405
Q

What do preventative cancer vaccines do?

A

Immunise agaisnt viruses known to cause cancer

406
Q

Name two viruses known to cause cancer?

A

Hep B - hepatocellular carcinoma

HPV - genital warts and cervical cancer

407
Q

In transplant rejection - which cells recognise foreign antigen?

A

T lymphocytes

408
Q

What class do all nucleated cells express on the cell surface?

A

Class I (HLA-A, HLA-B, HLA-C)

409
Q

What class do specialised antigen-presenting cells also express?

A

Class II (HLA-DR, HLA-DQ, HLA-DP)

410
Q

What do polymorphisms in HLA molecules maintain?

411
Q

In preventing transplant rejection what is it good to maximise?

A

The similarity between recipient and donor HLA

412
Q

What is the hierarchy of HLA-DR, HLA-A, HLA-B?

A

HLA-DR&raquo_space; HLA-B > HLA-A

413
Q

What is the difference between donor and recipient expressed as a number of?

A

Mismatches at HLA-A, HLA-B and HLA-DR

414
Q

Name two organs where HLA matching is used to allocate donor?

A
  1. Stem cell transplanatation - major preventable cause of graft versus host disease
  2. Kidney transplantation
415
Q

Give 3 organs where HLA matching is not used to allocate donor?

A

Lung
Heart
Liver

416
Q

In transplant rejection - once the resting T cell has met an antigen presenting cell, what occurs?

A

Activation of T cell, release of IL-2 and proliferation

417
Q

Give 4 effector functions of activated T cells?

A

Produce cytokines
Provide help to activate CD8 cells
Provide help for antibody production
Recruit phagocytic cells

418
Q

In acute cellular rejection - what cells recognise fonor antigens?

419
Q

One CD4 cells have recognised donor antigens and become acitvated - what occurs?

A

Production of cytokines: help for CD8 cells, help fopr B cells and recruitment and activation of macrophages and neutrophils

420
Q

What type of hypersensitivity response is acute cellualr rejection?

A

Type IV - delayed response

421
Q

What are the three methods of killing - CD8+ lymphocytes?

A

Release of toxins to kill target
Punch holes in target cells
Apoptotic cell death

422
Q

Name a toxin found in CD8 T cells that kills targets?

A

Granzyme B

423
Q

During mobilsation of phagocytes by activated CD4+ cells, what four functions do they perform?

A

Phagocytosis
Release of proteolytic enzymes
Production of cytokines
Production of oxygen radicals and nitropgen radicals

424
Q

What do T cells provide to activate B cells?

A

Costimulatory signals and cytokines

425
Q

What cells produce antibodies agaisnt graft antigens?

426
Q

What 4 things result form antibody production?

A

Complement activation
Opsonisation
Activation of NK cells
Recruitment of phagocytes

427
Q

In acute cellular rejection, what do biopsies show?

A

Infiltration of phagocytes (60%), T cells (30-40%), B cells and NK cells

428
Q

In acute cellular rejection - what activation markers do T cells express?

429
Q

In acute cellular rejection - what cytokines are increased?

A

IL-2 and gIFN

430
Q

What 3 other substances can be sued to assist diagnosis of acute cellular rejection?

A

Fas L
Granzyme
Perforin

431
Q

What are 3 broad symptoms of acute cellular rejection?

A

Deteriorating graft function
Pain and tenderness over graft
Fever

432
Q

In relation to deteriorating graft function - what 3 clinical changes occur with a kidney transplant?

A

Increased creatinine
Fluid retention
Hypertension

433
Q

In relation to deteriorating graft function - what 2 clinical changes occur with liver transplants?

A

Rise in LFTs

Coagulopathy

434
Q

In relation to lung transplants and deteriorating graft function - what 2 clinical changes occur?

A

Breathlessness

Pulmonary infiltrate

435
Q

What are the 4 types of transplant rejection?

A
  1. Hyperacute rejection
  2. Acute cellular rejection
  3. Acute vascular rejection
  4. Chronic allograft failure
436
Q

Give the timeframe, pathology, mechanism and treatment for hyperacute rejection

A

Minutes to hours
Thrombosis and necrosis
Preformed antibody and complement fixation
None

437
Q

Give the timeframe, pathology, mechanism and treatment for acute cellular rejection

A

5-30 days
Cellular infiltration, type IV hypersensitivity
CD4 and CD8 T cells
Immunosuppresion

438
Q

Give the timeframe, pathology, mechanism and treatment for acute vascular rejection

A

5-30 days
Vasculitis
T lymphocytes and antibody
Immunosuppression

439
Q

Give the timeframe, pathology, mechanism and treatment for chronic allograft failure

A

> 30 days
Fibrosis, scarring
Immune and non-immune mechanisms
Minimise drug toxicity, hypertension, hyperlipidaemia

440
Q

What is hyperacute rejection and what is it mediated by?

A

Rapid destruction of graft within minutes-hours

Mediated by pre-formed antibodies that react with donor cells

441
Q

Why would an individual have preformed antibodies agaisnt donor cells?

A

Blood group antigens - naturally occuring pre-formed antibodies, bind surface molecules found ubiquitously

HLA antigens - Arise through previous exposure (transplantation, transfusion, pregnancy), class I antibodies particularly important

442
Q

Patient has red cells expressing A and serum with natural anti-B antibodies, Heart transplant from group B: Where do the circulating preformed recipient anti-B antibodies bind to B blood group antigens?

A

On donor epithelium

443
Q

Patient has red cells expressing A and serum with natural anti-B antibodies, Heart transplant from group B: once antibodies have binded to antigens on donor epithelium complement is activated, what 3 things does complement activation cause?

A

Complement mediated lysis
Opsonisation
Increased permeability

444
Q

Patient has red cells expressing A and serum with natural anti-B antibodies, Heart transplant from group B: As well as the antibodies activating complement, what 2 other things happen?

A

Other cells rapidly recruited - phagocytes

Disruption of endothelium - platelets activated, infalmamtion, thrombosis

445
Q

What will transplantation of any donor tissue across incompatible blood groups result int>?

A

Hyperacute rejection

446
Q

As well as contradicting blood groups, what else can the recipient have that could cause a hyperacute rejection?

A

anti-donor HLA antibodies

447
Q

What type of transplant rejection are thrombosis of graft, often with neutrophil infiltrate and widespread necrosis and irretrievable graft loss clinical features of?

A

Hyperactue rejection

448
Q

In what two ways can hyperacute rejection be prevented?

A
  1. ABO matching- standard ABO blood typing, blood group cross match
  2. Identify if recipient has any anti-HLA antibodies - screening, HLA crossmatching
449
Q

What directly tests if serum from recipient is able to bind and/or to kill donor lymphocytes?

A

Crossmatching between donor and recipient

450
Q

What type of rejection is a rejection mediated predominantly by antibody?

A

Vascular rejection

451
Q

What type of transplant rejection is a major cause of graft loss and affects 50% of heart transplants in hte first 12 months?

A

Chronic allograft failure

452
Q

Give 5 non-immune risk factors for chronic allograft failure?

A
  1. Non compliance with medication
  2. hypertension
  3. Hyperlipidaemia
  4. Older donor age
  5. Calcineurin inhibitors (Ciclosporin, Tacrolimus)
453
Q

What are the three pathophysiological stages of chronic allograft failure?

A
  1. Cellular proliferation of smooth muscle of vessel wall
  2. Occlusion of vessel lumen
  3. Interstitial fibrosis and scarring is common
454
Q

Give three major complications of long-term immune suppression?

A

Infection
Malignancy
Atherosclerosis

455
Q

What in relation to the gums is a complication of ciclosporin?

A

Gingival hypertrophy

456
Q

What vaccines should never be given to immunosuppressed individuals?

A

Live vaccines

457
Q

What encourages you to think about immune deficiency?

A

SPUR infections

Resp JL (27 decks)

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