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Immunology Flashcards

1
Q

what are the consequences of immune recognition

A

intended destruction of the antigen and incidental tissue damage

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2
Q

what are hypersensitivity reactions

A

immune response that results in bystander damage to the self

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3
Q

briefly describe the 4 types of hypersensitivity reaction

A

I: immediate killing
II: Direct cell killing
III: Immune complex mediated
IV: delayed type hypersensitivity

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4
Q

describe what type 1 reactions are characterised by (immediate killing)

A

characterised by greatly enhanced sensitivity to normally innocuous substances, leading to physiological responses and tissue damage

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5
Q

what do type I reaction leads to

A

allergic diseases and reaction, signs and symptoms

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6
Q

define an allergy in immunological terms

A

IgE-mediated antibody response to external antigen (allergen)

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7
Q

what is happening to the prevalence of allergies

A

increasing

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8
Q

what are common allergens that trigger allergic reactions

A

(most soluble proteins that function as enzymes)- dust mites, protein in animal saliva, pollen, food, drugs, bee and wasp venom

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9
Q

are all adverse reactions allergic

A

no

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10
Q

what is the hygiene hypothesis

A

changes in microbial stimuli influence the maturation of the immune response- results in increased predisposition to allergic conditions during childhood

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11
Q

why is allergy prevalence increasing and associated with western countries

A

the hygiene hypothesis- improved sanitation and decreased incidence of infectious disease

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12
Q

what enhances the immune system

A

exposure to bacteria or viruses e.g. infections contracted from siblings or peers at daycare centres

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13
Q

what happens to CD4 T lymphocytes at birth

A

primed to develop into TH2 cells

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14
Q

what does exposure to antigens do to the development of TH2 cells

A

dampens it, creating bias towards TH1 response- TH1 differentiation stimuli, limiting allergies and asthma

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15
Q

what are the generic features of type 1 allergic disease

A

occurs quickly after exposure to allergen (1-2 hours), reactions influenced by site of contact

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16
Q

what are the specific features of type 1 allergic disease

A

asthma, urticaria (hives), angioedema (swelling), allergic rhinitis (hayfever), allergic conjunctivitis, diarrhoea and vomiting, anaphylaxis

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17
Q

what immune cells are involved in allergic disease

A

b lymphocytes, t lymphocytes, mast cells (eosinophils and basophils)

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18
Q

how are B lymphocytes involved in allergic reactions

A

recognise antigen, produce antigen-specific IgE antibodies

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19
Q

how are T lymphocytes involved in allergic reactions

A

TH2 cells provide help for B lymphocytes to make IgE antibody

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20
Q

how are mast cells involved in allergic reactions

A

inflammatory cells that release vasoactive substances

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21
Q

what results in the differentiation of CD4 T cells into effector TH2 cytokine-producing cells

A

stimulation of allergen-specific T cells by allergen-derived peptides, presented by dendritic cells in the context of class II MHC molecules

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22
Q

what regulate the immune response

A

interleukins IL-4, IL-13, IL-5 produced by TH2 cells

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23
Q

how do interleukins regulate the allergic response (3)

A

regulate the synthesis of IgE by B cells, stimulate differentiation and migration of eosinophils from the bone marrow into the blood, helping to activate mast cells and eosinophils at sites of allergen exposure

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24
Q

how is the secretions of B cells altered by TH2

A

initially secrete IgM but initiated by TH2 cells to produce IgE

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25
Q

what does mast cells role in orchestrating the inflammatory cascade lead to

A

lead to increase blood flow, contraction of smooth muscle, increase vascular permeability and secretions at mucosal surfaces

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26
Q

what vasoactive substances do mast cells produce that drive acute inflammation

A

histamine, leukotrienes, pro-inflammatory cytokines including IL-4 and TNF-alpha

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27
Q

what do mast cells express on their surface

A

receptors for the FC region of IgE antibody on their surface

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28
Q

what happens to B cells on first encounter with allergen

A

produce antigen-specific IgE antibody

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29
Q

what happens to residual IgE antibodies

A

bind to circulating mast cells via Fc receptors

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30
Q

what is the region of the antibody that is specific to the antibody and where it binds to Fc receptors

A

the heavy chain

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31
Q

what happens when allergens re-encounter the IgE- coated mast cells

A

cell membrane disrupted and vasoactive mediators (histamine, tryptase) released. also increased cytokine and leukotriene transcription

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32
Q

what is atopic asthma

A

allergic disease in the lungs

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33
Q

is non allergic asthma IgE mediated

A

no

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34
Q

describe intrinsic asthma

A

non allergic

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35
Q

describe extrinsic asthma

A

response to external allergen, IgE-mediated

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36
Q

what happens clinically when histamine and other inflammatory mediators are released

A

muscle spasm, mucosal inflammation, inflammatory cell infiltrate

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37
Q

what are pro inflammatory mediators

A

pro inflammatory cytokines

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38
Q

what does muscle spasm lead to

A

bronchoconstriction; wheeze

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39
Q

what does mucosal inflammation lead to

A

mucosal oedema, increases secretions; sputum production

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40
Q

what does inflammatory cell infiltrate lead to

A

infiltration of lymphocytes and eosinophils into bronchioles; sputum (often yellow) (associated with chronicity)

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41
Q

what happens clinically when an allergic reaction happens in the lung

A

increased; mucous production, swelling, breathing rate

in and expiratory wheeze

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42
Q

what happens in degranulation of mast cells

A

when antimicrobial/cytotoxic molecules release from mast cells, when membrane disrupted

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43
Q

what is urticaria and how long does it last

A

hives, 2-6 (occasionally 24) hours

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44
Q

what is angioedema

A

self-limited, localised swelling of subcutaneous tissues or mucous membranes (non-pitting oedema

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45
Q

what are the clinical features of anaphylaxis

A

feeling of impending doom, loss of consciousness, death, angioedema of lips of mucosal membrane, stridor, laryngeal obstruction, hypotension, oral itching, vomiting, diarrhoea, abdominal pain, conjunctival infection, wheeze, bronchoconstriction, itch in palms, soles of feet and genitalia

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46
Q

why is it important to diagnose allergic disease (4)

A

confirm diagnosis, identify causative agents, determine risk of future severe reaction, determine appropriateness of therapy

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47
Q

name an elective investigation used to diagnose allergic disease

A

skin prick test

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48
Q

how can allergic disease be diagnosed during anaphylactic episode

A

evidence of mast cell degranulation (blood test- serum mast cell tryptase levels)

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49
Q

what is the first step in management of IgE mediated allergic disorders

A

indentification of triggers (skin prick test, clinical history, specific IgE tests) and avoidance of allergens

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50
Q

how is mast cell activation blocked?

A

via mast cell stabilisers (sodium cromoglycate)- stabilises mast cell membranes, prevents release of inflammatory mediators

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51
Q

what prevents the effects of mast cell activation

A

anti-histamines, leukotriene receptor antagonist

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52
Q

how do anti-histamines work

A

anti-histamines- block biological effects of histamines , used prophylactically (preventative) and to control symptoms (e.g. loratadine and cetirizine)

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53
Q

how do leukotriene receptor antagonists work

A

block effects of leukotrienes which are synthesised by mast cells after activation (e.g. montelukast)

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54
Q

name and describe the anti-inflammatory agents used to treat IgE mediated disorders

A

corticosteriods, inhibits formation of many different inflammatory mediators

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55
Q

how is anaphylaxis treated

A

self-injectable adrenalin- acts on beta2 adrenergic receptors to constrict atrial smooth muscle

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56
Q

what does the constriction of arterial smooth muscle because of adrenalin result in

A

increased blood pressure, limited vascular leakage, dilation of bronchial smooth muscle, decreasing airflow obstruction

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57
Q

describe immunotherapy

A

controlled exposure to increasing amounts of allergen- gradually increasing dose of subcutaneous injection

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58
Q

summarise the ways in which IgE mediated allergic disorders are managed

A

avoidance of allergen, block mast cell activation, prevent effects of mast cell activation, anaphylaxis management, immunotherapy

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59
Q

what cam mast cells with IgE antobodies lead to, especially in asthma

A

activation of eosinophils

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60
Q

what is the atopic trait that is inherited and found in people who suffer unduly from allergy

A

raised levels of IgE

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61
Q

what can mast cell degranulation be triggered by

A

tissue injury, complement activation and by some bacteria independently of IgE, IgE

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62
Q

which allergens which lead to built in immune response now useless are one of the most powerful

A

worm allergens

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63
Q

classify type II hypersensitivity reactions

A

direct cell killing (cell bound antigen)

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64
Q

what does an antibody bind to

A

cell-surface antigens

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65
Q

what does antigen binding result in

A

activation of complement (cell lysis (rupture)) and opsonisation (antibody-mediated phagocytosis)

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66
Q

what does the heavy chain region of the antibody trigger

A

immune response

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67
Q

what antigen initiates the classical activation pathway of complement

A

IgM

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68
Q

how does IgM initiate complement

A

engages multiple antigens on surface of cell which causes conformational change in FC region of antibody

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69
Q

what do phagocytes express on their surface to bond to the heavy chain

A

Fc receptors for IgG

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70
Q

what part of antibody neutralises

A

antigen binding portion

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71
Q

what are the functions of antibodies (6)

A

activates complement, activates antigens dependant cellular activity (e.g. NK cells/ eosinophils), acts on opsonins, activates T lymphocytes, causes antigen clumping and inactivation of bacteria complexes, triggers mast cell degranulation

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72
Q

describe complement

A

20 tightly regulated, linked proteins produced by liver in response to inflammation

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73
Q

what happens when complement is activated

A

enzymatically activate other proteins in a biological cascade

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74
Q

what antibody is best for parasitic infection

A

IgE

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75
Q

for normal antigens what antibody is the best

A

IgG

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76
Q

what does IgG trigger

A

classical pathways and cleavage of C3 into C3a and C3b, and then goes downstream into many different functions

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77
Q

what are the three pathways that lead ti activation of complement (C3)

A

classical- antigen + antibody + C1 complex
Lectin- MBL + mannose(+ve) pathogen
alternative- spontaneous C3 cleavage

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78
Q

what does the cleavage of C3 lead to

A

chemotaxis, solubilzation, direct killing (membrane attack complex), opsonisation

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79
Q

what does membrane attack complex do

A

punches holes in bacterial cell membranes, can directly kill encapsulated bacteria

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80
Q

what molecules involved in chemotaxis increase permeability of blood vessels

A

C3a and C5a fragments

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81
Q

why in chemotaxis is permeability of blood vessels increased

A

to increase traffic to cells of sites of infection

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82
Q

what do opsonins act as a bridge between

A

the pathogen and phagocyte receptors

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83
Q

what fragment of C3 is an opsonin

A

C3b

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84
Q

how is complement regulated via negative feedback

A

as fragments of complement can dissolve the immune complexes which triggered them- switching off complement activation

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85
Q

what is solubilzation

A

complement proteins breaking down large antibody complexes so they can be phagocytosed

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86
Q

give a clinical example of type II hypersensitivity

A

immune haemolytic amaemias

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87
Q

what type of hypersensitivity reaction is ALLERGIC haemolytic anaemia

A

type I

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88
Q

what happens in an acute haemolytic transfusion reaction

A

ABO incompatibility = lysis of donor erythrocytes by pre-formed IgG antibodies

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89
Q

what are the symptoms of a transfusion reaction

A

increased resp rate, tachycardia, kidney problems, decreased O2 sats

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90
Q

what type of antibodies are in type A blood

A

anti B

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91
Q

what type of antibodies are in type B blood

A

anti A

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92
Q

what type of antibodies are in type AB blood

A

neither A/B

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93
Q

what type of antibodies are in type O blood

A

both anti A & B

94
Q

describe penicillin induced immune haemolytic anaemia

A

penicillin too small to induce antibodies, binds to surface protein of red blood cells, now has very antigenic structure, drives B cell response which leads to production of IgG antibodies and immune response (antibody bond to FcR om specific macrophage)

95
Q

how is a transfusion reaction treated

A

plasmapheresis- removal of pathogenic antibodies from serum in the blood via cell separator
immunosupression-switch off B cell production

96
Q

classify type III hypersensitivity reactions

A

immune complex needed

97
Q

what is the key component of a type III reaction

A

antibody to soluble antigens

98
Q

describe the pathophysiology of type III reactions

A

in presence of excess antigen, antibody binds forming small immune complexes which are trapped in small blood vessels, joints and glomeruli (filtration unit of kidney)

99
Q

what do the signs and symptoms of type III reactions depend on

A

the tissue in which the immune complex is deposited in

100
Q

what does the presence of an immune complex in type III reactions cause

A

activation of complement and attracts inflammatory cells (neutrophils). Neutrophils cannot phagocytose complex due to size so degranulate releasing enzymes causing bystander tissue damage

101
Q

why do mould particles cause type III reactions

A

as when inhaled stimulate antibody formation- antibodies form immune complexes with antigen; results in complement activation, inflammation, recruitment of other leukocytes

102
Q

give examples of acute hypersensitivity pneumonitis

A

farmers lung, bird fanciers lung, cheese workers lung, malt workers lung

103
Q

what is ACUTE hypersensitivity pneumonitis mediated by

A

type III hypersensitivity response- when immune complexes deposited in walls of alveoli and bronchioles- CHRONIC DIFFERENT

104
Q

what are the symptoms of acute hypersensitivity pneumonitis

A

wheeze, breathlessness, malaise and pyrexia

105
Q

explain wheezing in A.H.P

A

bronchoconstriction- inflammation of the terminal bronchioles and alveoli caused by activated phagocytes and complement

106
Q

explain breathlessness in A.H.P

A

alveolitis, inflammation of air sacs, caused by activated phagocytes and complement

107
Q

explain malaise and pyrexia in A.H.P

A

systemic manifestation of inflammatory response

108
Q

how are type III hypersensitivity reactions managed

A

avoidance, corticoidsteriods (decrease inflammation), immunosuppression (decrease production of IgG)

109
Q

what type of drug are corticosteroids

A

glucocorticoids

110
Q

classify type IV hypersensitivity reactions

A

delayed type hypersensitivity

111
Q

what are type IV hypersensitivity reactions driven by

A

CD4 + t cells

112
Q

what disease are associated with type IV hypersensitivity reactions

A

autoimmune- type 1 diabetes, psoriasis, rheumatoid arthritis
non-autoimmune- contact dermatitis, TB, leprosy, sarcoidosis, cellular rejection of organ transplant

113
Q

what initially happens in a type IV reaction

A

generation of primed effector TH1 cells and memory T cells

114
Q

what happens after subsequent exposure to pathogen in a IV response

A

activation of previously primed t cells, recruitment of macrophages, other lymphocytes, neutrophils… release of proteolytic enzymes, presistent inflammation- tissue damage

115
Q

when does subsequent exposure in IV reactions happen

A

in autoimmunity, constant exposure to antibody or antigen stimulates macrophages which drives an ongoing response

116
Q

give an example of type IV hypersensitivity reaction

A

poison ivy

117
Q

what is chemokines role in type IV reactions

A

macrophage recruitment to site of antigen

118
Q

what is INF-gamma role in type IV reactions

A

activates macrophages, increasing release of inflammatory mediators

119
Q

what is INF-alpha and leukotriene role in type IV reactions

A

local tissue destruction, increased expression of adhesion molecules on local blood vessels

120
Q

what are granulomas and their role

A

collection of walled off macrophages and T helper 1 cells to encase inflammatory reaction and limit tissue damage

121
Q

e.g of type IV hypersensitivity- sarcoidosis, describe

A

multisystem granulomatous disease, characterised by granulomas, patches of red and swollen tissue

122
Q

describe the immune process of sarcoidosis

A

antigen inhaled, stimulation of alveolar macrophages and CD4 and CD8 t cells, and B cells. Failure to clear antigen results in persistent inflammation and granuloma formation= tissue damage and fibrosis

123
Q

how is sarcoidosis managed

A

systemic corticosteroids- block t cell and macrophage activation

124
Q

what is the ABCD of hypersensitivity reactions

A 
I= Aleergic Anaphylaxis Atropy 
II= antiBody 
III= immune Complex
IV= Delayed
125
Q

describe the onset of type 1 reactions

A

seconds if IgE preformed

126
Q

describe the onset of type 2 reactions

A

seconds if IgG/M preformed

127
Q

describe the onset of type 3 reactions

A

hours if IgG preformed

128
Q

describe the onset of type 4 reactions

A

2-3 days

129
Q

describe the infectious trigger of type 1 reactions

A

parasites

130
Q

describe the infectious trigger of type 2 reactions

A

none

131
Q

describe the infectious trigger of type 3 reactions

A

post streptococcal glomeruonephritis

132
Q

describe the infectious trigger of type 4 reactions

A

Hep B virus

133
Q

describe the environmental trigger of type 1 reactions

A

allergens

134
Q

describe the environmental trigger of type 2 reactions

A

immune haemolytic anaemias

135
Q

describe the environmental trigger of type 3 reactions

A

farmers lung

136
Q

describe the environmental trigger of type 4 reactions

A

contact dermatitis, sarcoidosis

137
Q

describe the adaptive immune mediators of type 1 reactions

A

TH2 cells, B cells, IgE

138
Q

describe the adaptive immune mediators of type 2 reactions

A

B cells, IgG/M

139
Q

describe the adaptive immune mediators of type 3 reactions

A

B cells IgG

140
Q

describe the adaptive immune mediators of type 4 reactions

A

Th1 cells

141
Q

describe the innate immune mediators of type 1 reactions

A

mast cells, eosinophils

142
Q

describe the innate immune mediators of type 2 reactions

A

complement, phagocytes

143
Q

describe the innate immune mediators of type 3 reactions

A

complement, neutrophils

144
Q

describe the innate immune mediators of type 4 reactions

A

macrophages

145
Q

what is the major hallmark of immune deficiency

A

recurrent infections; serious, persistent, unusual- SPUR

146
Q

what other features suggest a immunodeficiency

A

weight loss, skin rash, diarrhoea, mouth ulcers

147
Q

what are the classifications of immunodeficiencies

A

primary (gentically encoded), secondary (acquired throughout life)

148
Q

what conditions are associated with secondary immune deficiency

A

infection, treatments, malignancy, biochemical and nutritional disorders, physiological immune deficiency

149
Q

what cause physiological secondary immune deficiencies

A

old age/ prematurity

150
Q

how can chemotherapy cause immune deficiency

A

as kills rapidly dividing cells- bone marrow heamatic stem cells

151
Q

what is the purpose of PAMPs

A

to recognise structures that are unique to infectious organisms

152
Q

what cells make up the innate immune system

A

macrophages, neutrophils, mast cells, natural killer cells

153
Q

what proteins make up the innate immune system

A

complement, acute phase proteins, cytokines

154
Q

what is tnf alpha

A

pro inflammatory mediator

155
Q

what proteins and cells make up the acquired immune response

A

B and T lymphocytes, proteins - antibodies

156
Q

name two phagocytes

A

macrophages and neutrophils

157
Q

what are clinical features of phagocyte deficiencies

A

recurrent infections of common and unusual sites

158
Q

what produces chemokine signals that initiate chemotaxis

A

pathogen or chemotactic signals from infected tissue

159
Q

what is recticular dysgenesis, how is it treated and what happens is it is not

A

immunodeficiency where production of immune cells is blocked, bone marrow transplant, die shortly after birth

160
Q

what is kotsmann syndrome and what causes it

A

blocked neutrophil development and maturation due to mutation in receptor or cytokine

161
Q

what is a SCID

A

severe combines immunodeficiencies

162
Q

what are SCID genetically and phenotypically

A

heterogeneous diseases

163
Q

describe kostmann syndrome genetically

A

rare autosomal recessive

164
Q

what is neutropenia

A

low levels of neutrophils in the blood

165
Q

what will affect the treatment of kostmann syndrome

A

whether the mutation is in the cytokine or the ligand

166
Q

how is kostmann syndrome treated

A

supportive treatments for the infections, definitive treatments (stem cell transplant and granulocyte colony stimulating factor

167
Q

what would you expect in someone whose phagocytes were unable to bind to endothelial adhesion molecules

A

recurrent infections, v high neutrophil blood count, no pus formation in infected sites

168
Q

why would there be an increased neutrophil blood level if phagocytes couldn’t bind to endothelial adhesion molecules

A

as cant migrate from vessels and cytokines would send signals to bone marrow to produce more

169
Q

describe leukocyte adhesion deficiency and how it is acquired

A

failure of mobilised neutrophils to recognise activation markers expressed on endothelial cells so can exit bloodstream- genetically inherited

170
Q

describe direct recognition of pathogens by phagocytes

A

done by pathogen recognition receptors that recognises microbial specific structures

171
Q

describe indirect recognition of pathogens by phagocytes

A

opsonins (e.g. antibody or complement fragment) act as binding enhancers for the process of phagocytosis and bind to phagocyte when also bound to pathogen

172
Q

what can cause a defect in pathogen recognition cause

A

defect in opsonin receptors (Fc or CR1)

173
Q

what investigations would be undertaken to determine whether a patient has an immunodeficiency

A

blood count- neutrophil numbers, serum immunoglobulins, IgE, complement

174
Q

what is chronic granulomatous disease

A

failure of oxidative killing mechanisms- deficiency in intracellular killing mechanisms of phagocytes

175
Q

what does failure of oxidative killing mechanisms result in

A

inability to generate oxygen free radicals- impaired intracellular killing of micro-organisms

176
Q

what is the presentation of a failure of oxidative killing mechanisms

A

inability to clear organisms- excessive inflammation (cant degrade and immune cells build up) and granuloma formation

177
Q

what are the clinical features of chronic granulomatous disease

A

recurrent deep bacterial infections, recurrent fungal infections, failure to thrive, lymphadenopathy, granuloma formation

178
Q

how is chronic granulmatous disease investigated

A

NBT test to show whether neutrophils can kill through production of oxidative free radicals

179
Q

how is chronic granulomatous treated

A

supportive treatment (prophylactic antibiotics/fungals) definitive treatment- stem cell transplant and gene therapy

180
Q

how does mycobacteria avoid the immune system

A

by hiding within immune cells- macrophages

181
Q

how does the body defend against intracellular organisms such as mycobacteria (TB)

A

activate IL-12- IFN gamma network ( infected macrophages stimulated to produce IL-12 which induces TH1 cells to secrete IFNgamma which stimulates production of TNF which stimulates oxidative pathways)

182
Q

what are defects in the IL-12; INFgamma axis associated with

A

susceptibility to intracellular bacteria- e.g mycobacterial infection (TB) and salmonella

183
Q

how is phagocyte function investigated

A

blood count, presence of pus, expression of neutrophils adhesion mols (Phagocyte recruitment) chemotactic and phagocytosis assays find and kill the bug) NBT test (oxidative killing)

184
Q

describe neutrophil count, pus formation, leukocyte adhesion markers and NBT test in congenital neutropaenia

A

NC-absent
PF-No
LAM-normal
NBT-absent (no neutrophils)

185
Q

describe neutrophil count, pus formation, leukocyte adhesion markers and NBT test in leukocyte adhesion defect

A

NC-increased during infection
PF-no
LAM-absent
NBT- normal

186
Q

describe neutrophil count, pus formation, leukocyte adhesion markers and NBT test in chronic granulomatous disease

A

NC-normal
PF-yes
LAM-normal
NBT-abnormal

187
Q

what does failure of neutrophil differentiation cause

A

severe congential neutropaenia and cyclic neutropaenia (recurrent)

188
Q

name the organs involved in the acquired immune system

A

lymphatics, bone marrow, intestines, spleen, kidney, appendix, thymus, heart

189
Q

where does proliferation and maturation of T lymphocytes happen

A

in the thymus

190
Q

what are the function of CD4 + T lymphocytes

A

immunoregulators and recognise peptides presented on MHC class 2 molecules

191
Q

what is the role of CD8+ T lymphocytes

A

cytotoxic cells- recognise peptides in association with MHC class 1 molecules and kill cells directly

192
Q

what are CD8+ T lymphocytes most important in protecting the body form

A

viral infections and tumours

193
Q

what is the function of B lymphocytes

A

antibody production and antigen presentation

194
Q

where do B lymphocytes arise from

A

haemopoetic stem cells in bone marrow

195
Q

where are mature B lymphocytes mainly found

A

bone marrow, lymphoid tissue, spleen

196
Q

what causes B lymphocytes to activate

A

encounters with antigen within lymph nodes

197
Q

what are 3 functions of antibodies

A

identification of pathogens, recruitment of other components of immune response (complement, pathogens, NK cells), neutralisation of toxins

198
Q

what is recticular dysgenesis

A

defect of the haemopoetic stem cells

199
Q

what is severe combined immunodeficiency a defect of

A

lymphoid precursor- failure of production of lymphocytes

200
Q

what is the clinical phenotype of severe combined immunodeficiency

A

unwell by 3 months of age, persistent diarrhoea, infections, unusual skin disease, family history of early infant death

201
Q

what is the most common form of severe combined immunodeficiency

A

X-linked SCID, mutation of a component of the IL-2 receptors

202
Q

what is the clinical phenotype of x-linked SCID

A

very low or absent T cells (as IL2 important for T cell development), normal or increased B cells, poorly developed lymphoid tissue and thymus

203
Q

how is SCID treated

A

prophylactic treatment for infections, definitive treatment- stem cell transplant, gene therapy (for ADA-SCID)

204
Q

what is DiGeorge syndrome

A

developmental defect of the 3rd/4th pharyngeal pouch- cumulative effect of deletion of several genes

205
Q

what is the clinical presentation of DiGeorge syndrome

A

hypocalcaemia, oesophageal atresia, T cell lymphopenia, congenital heart disease, facial deformities

206
Q

what causes recurrent viral infections

A

CD8 + T cells absent (+other T cells)

207
Q

what causes recurrent bacterial infections

A

lack of CD4 + TFH as help B cells make antibody

208
Q

what causes recurrent fungal infections

A

lack of CD4 + TH cells

209
Q

what will laboratory investigations show in a patient with DiGeorge Syndrome

A

absent or decreased T cells (as defective activation response), normal or increased B cells, low serum antibody levels, normal NK levels

210
Q

how is DiGeorge syndrome managed

A

correct metabolic/ cardiac abnormalities, prophylactic antibiotics; T cell function improves with age

211
Q

what part of T lymphocyte development does DiGeorge syndrome affect

A

export of mature T lymphocytes to periphery

212
Q

what are disorders of T cell effector function

A

cytokine production, cytotoxicity, T-B cell communication

213
Q

a deficiency in a component in what axis can lead to a deficiency of cytokine production by T cells

A

IL-12; IFN gamma network

214
Q

in what form of SCID is normal T cells level present

A

bare lymphocyte syndrome

215
Q

what does bare lymphocyte syndrome result in

A

failure of expression of MHC molecules

216
Q

what does autoimmune lymphoproliferative syndrome result in

A

failure of abnormal apoptosis

217
Q

what are the clinical features of T cell deficiencies

A

recurrent infection, autoimmune disease, malignancies at young age

218
Q

what is affected when there is an immune deficiency affecting B lymphocytes

A

antibody deficiencies

219
Q

what is the presentation of antibodies deficiencies

A

recurrent bacterial infections, antibody mediated autoimmune diseases

220
Q

what is brutons x-linked hypogammaglobulinaemia

A

b cell maturation defect

221
Q

what does common variable immune deficiency affect and result in

A

B cells- failure to produce IgG, failure of lymphocyte precursors,

222
Q

what is common variable immune deficiency and its clinical features

A

heterogeneous group of disorders; reccurent bacterial infections, autoimmune and granulomatous disease

223
Q

what is x-linked hyper IgM syndrome

A

failure of TFH cell co-stimulation

224
Q

what is selective antibody deficiency

A

failure to produce specific antibodies

225
Q

what are the clinical features of B cell deficiencies

A

recurrent infections, opportunistic infections, antibody mediated autoimmune disease

226
Q

how are B cell deficiencies managed

A

treatment of infection, immunoglobulin replacement, stem cell transplantation

227
Q

out of IgM, IgG and IgA what serum immunoglobulins will be normal in selective IgA deficiency

A

M and G, A absent or decreased

228
Q

out of IgM, IgG and IgA what serum immunoglobulins will be normal in x linked agammaglobulinaemia

A

none

229
Q

out of IgM, IgG and IgA what serum immunoglobulins will be normal in common variable immune deficiency

A

IgG, M and A decreased or absent

230
Q

out of IgM, IgG and IgA what serum immunoglobulins will be normal in specific antibody deficiency

A

all

231
Q

in which primary antibody deficiency are b lymphocyte sub populations decreased or absent

A

x linked agamma-globulinaemia

232
Q

what other investigation can be used to investigate primary antibody deficiencies

A

failure to produce vaccine specific antibodies (happens in all except selective IgA deficiency

Respiratory (26 decks)

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