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Path (hopefully more condensed) > Immuno > Flashcards

Immuno Flashcards

1
Q

infant with diarrhoea, failure to thrive and skin infections (candida). atrophy of thymus and mucosa-associated lymphoid tissue noted. Diminished T cells, non-functional b cells

A

Severe combined immunodeficiency

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2
Q

child with recurrent bacterial infections. blood count shows neutropenia. NBT test is normal

A

Kostmann syndrome

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3
Q

autosomal dominant condition with neutropenia every 3 weeks or so

A

cyclic neutropenia

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4
Q

condition with a very high neutrophil count, but absent pus formation. NBT test positive. Delayed umbilical cord sloughing

A

leukocyte adhesion deficiency

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5
Q

child with recurrent chest infections. normal t cell count, but diminsished b cell count, as well as IgA, IgM and IgG. Absent lymph nodes and tonsils. B cells cannot mature

A

Bruton’s x-linked a-gammaglobulinaemia

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6
Q

patient with diarrhoea, fever, tiredness. chest infections since diarrhoea, Crohn’s history

A

protein-losing enteropathy

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7
Q

child with recurrent chest infections, cleft lip, hypocalcaemia. Reduced T cells, IgG, IgA, absent thymus. Normal B cells, IgM. Also cardiac abnormalities and oesophageal atresia.

A

Di George’s syndrome

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8
Q

Elevated IgM, no IgG, IgA or IgE

A

Hyper IgM syndrome

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9
Q

child with recurrent chest infections and eczema. also easy bruising, nose bleeds and gi bleeds,reduced IgM, normal IgG, elevated IgA and IgE

A

Wiskott-Aldrich syndrome

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10
Q

20 year old with recurrent pneumonia. predisposed to autoimmune diseases, as well as increased risk of lymphoma and granulomas. MHC class 3 mutation. Reduced B and T cell functionality, reduced IgM, IgA, IgG and IgE.

A

Common variable immunodeficiency

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11
Q

Child recurrent skin infections and fungal infections. Chronic inflammation with non-caseating granulomas. NBT test negative. Lymphadenopathy, hepatosplenomegaly, pus. Normal neutrophil count. NADPH oxidase deficiency.

A

Chronic granulomatous disease

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12
Q

Sclerosing cholangitis with hepatomegaly and jaundice. MHC 1 deficiency –> CD8 T cell deficiency

A

Type 1 bare lymphocyte syndrome

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13
Q

Sclerosing cholangitis with hepatomegaly and jaundice. MHC 2 deficiency –> CD4 T cell deficiency

A

Type 2 bare lymphocyte syndrome

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14
Q

Recurrent mild resp/ GI infections, anaphylaxis to transfusions

A

Selective IgA deficiency

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15
Q

Sickle cell anaemia patients are vulnerable to which type of infection

A

Encapsulated bacteria (S. pneumonia)

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16
Q

Autosomal recessive condition resulting in severe sepsis a few days after birth. Absolute deficiency in both granulocytes and lymphocytes

A

Reticular dysgenesis

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17
Q

ADA deficiency cell count abnormalities

A

Low B, T and NK cells

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18
Q

Disease resulting in reduced activation of macrophages, so no granulomas can form. Patient susceptible to intracellular pathogens (TB, salmonella)

A

Interferon gamma receptor deficiency

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19
Q

Most important HLA antigens to match

A

HLA-A, HLA-B, HLA-DR

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20
Q

Name a monogenic auto-inflammatory disease

A

Familial mediterranean fever

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21
Q

Pathogens you are susceptible to in chronic granulomatous disease

A

catalase positive (pseudomonas, listeria, aspergillus, candida, E. coli, s. aureus, serratia)

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22
Q

What do all patients with C2 deficiency have

A

SLE

severe skin disease, increased infections

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23
Q

Patients with increased susceptibility to infection in MBL deficiency

A

premature infants, chemotherapy, HIV, Ab deficiency

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24
Q

alternative complement pathway deficiencies lead to increased infections with…

A

encapsulated bacteria

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25
Q

SLE pathogenesis

A

production of immune complexes leading to consumption of C3 and C4

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26
Q

Ix for classical pathway activity

A

CH50

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27
Q

Ix for alternative pathway activity

A

AP50

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28
Q

factors of classical complement pathway

A

C1q, C1r, C1s, C2, C4

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29
Q

most common classical pathway deficiency

A

C2

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30
Q

common pathway factors

A

C3, C5-9

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31
Q

deficiency of common pathway factors leads to infections with…

A

N. meningitidis

S. pneumoniae

H. influenzae

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32
Q

5 month old girl with recurrent fungal infections, diarrhoea and failure to thrive. Family history of early infant death. Bloods show absent B cells, T cells and NK cells

A

ADA deficiency

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33
Q

What protects SCID infant for first 3 months

A

maternal IgG

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34
Q

autoinflammatory conditions are a defect in which type of immunity

A

innate

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35
Q

autoimmune conditions are a defect in which type of immunity

A

adaptive

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36
Q

familial mediterranean fever symptoms

A

periodic fevers and serositis

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37
Q

familial mediterranean fever pathogenesis

A

MEFV mutation –> abnormal TNFa/ IL1 signalling

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38
Q

familial mediterranean fever treatment

A

colchicine

anakinra (IL1-R antagonist)

etanercept (TNFa inhibitor)

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39
Q

name a polygenic autoinflammatory disease

A

crohn’s

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40
Q

name a mixed pattern disease

A

Ankylosing spondylitis

psoriatic arthritis

behcet’s

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41
Q

mixed pattern disease pathogenesis

A

HLA-B27 mutation affects CD8 T cells, Th17 cells, NK cells

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42
Q

name a polygenic autoimmune disease

A

rheumatoid arthritis, myasthenia gravis, pernicious anaemia, SLE

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43
Q

Goodpasture disease HLA association

A

HLA-DR15

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44
Q

Grave’s disease HLA association

A

HLA-DR3

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45
Q

SLE HLA association

A

HLA-DR3

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46
Q

T1DM HLA association

A

HLA-DR3/4

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47
Q

Rheumatoid arthritis HLA association

A

HLA-DR4

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48
Q

Name a monogenic autoimmune disease

A

APECED

IPEX

ALPS

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49
Q

APECED features

A

mutation of AIRE transcription factor means lack of negative T cell selection

autoimmune polyendocrinopathy candidiasis ectodermal dystrophy

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50
Q

IPEX features

A

mutation in FoxP3 required fpr T reg cell development

Treg cells CD25+, FoxP3x

immune dysregulation, polyendocrinopathy, enteropathy, X-linked

diarrhoea, diabetes, dermatitis

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51
Q

ALPS features

A

mutation in Fas pathway –> T cells not killed in thymus

large spleen + lymph nodes, autoimmune cytopenia, lymphoma

autoimmune lymphoproliferative syndrome

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52
Q

type 1 hypersensitivity mechanism

A

sensitisation in primary exposure

Ag cross links IgE on mast cells –> degranulation

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53
Q

type 1 hypersensitivity example

A

atopic eczema

anaphylaxis

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54
Q

type 2 hypersensitivity mechanism

A

antibody binds to surface antigen

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55
Q

type 2 hypersensitivity example

A

Graves, myasthenia gravis

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56
Q

type 3 hypersensitivity mechanism

A

antibody-antigen complexes deposit in tissue

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57
Q

type 3 hypersensitivity example

A

SLE, serum sickness

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58
Q

type 4 hypersensitivity mechanism

A

T cell mediated

CD4+ release cytokines
CD8+ kill target cells

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59
Q

type 4 hypersensitivity example

A

T1DM, contact dermatitis

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60
Q

live attenuated vaccines examples

A

MMR (HIV safe)

Varicella zoster

BCG (NOT HIV safe)

Yellow fever (NOT HIV safe)

oral polio, typhoid

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61
Q

inactivated vaccine example

A

polio (salk)

anthrax

influenza

hep a

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62
Q

component/ subunit vaccine example

A

Hep B

HPV

Influenza

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63
Q

Toxoid vaccine example

A

diptheria

tetanus

pertussis

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64
Q

conjugate vaccine example

A

N. meninigitidis

Haemophilus influenza B

Strep pneumoniae

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65
Q

DNA/ mRNA vaccine example

A

covid vaccines

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66
Q

human normal immunoglobulin uses

A

ITP, Kawasaki, GBS, measles

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67
Q

what is pavilizumab

A

monoclonal antibody for RSV

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68
Q

interferon alpha uses

A

Hep B, C and CML

Kaposi’s sarcoma

hairy cell leukaemia

multiple myeloma

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69
Q

interferon beta uses

A

behcet’s

relapsing MS

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70
Q

interferon gamma use

A

chronic granulomatous disease

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71
Q

which antibody is responsible for protecting against GI infections

A

IgA

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72
Q

ipilimumab receptor and indication

A

CTLA4

melanoma

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73
Q

moromonab receptor and indication

A

CD3

transplant rejection

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74
Q

dacilizumab receptor and indication

A

IL2

transplant rejection

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75
Q

denosumab receptor and indication

A

RANKL

osteoporosis, bone mets pain, hypercalcaemia

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76
Q

infliximab receptor and indication

A

TNF-alpha

IBD

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77
Q

how do corticosteroids cause immunosuppression

A

reduce traffic of neutrophils to sites (causes transient increase in plasma counts)

reduce phagocytosis and release of proteolytic enzymes

increase lymphocyte apoptosis

increase lymphocyte migration back into nodes, spleen etc

inhibit NF-kappaB

inhibit phospholipase A2

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78
Q

azathioprine MOA

A

stops DNA production (metabolised to purine analogue 6-mercaptopurine)

affects T cells more than B

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79
Q

azathioprine indication

A

transplantation

autoimmune and autoinflammatory diseases

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80
Q

mycophenolate MOA

A

blocks de novo guanosine synthesis

affects T cells more than B cells

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81
Q

mycophenolate indication

A

transplantation

autoimmune and vasculitis

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82
Q

mycophenolate side effects

A

HSV reactivation

JC virus reactivation –> progressive multifocal leukoencephalopathy (PML)

progressive neural demyelination condition

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83
Q

cyclophosphamide MOA

A

alkylates gaunine base –> stops DNA replication

affects B cells more than T cells

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84
Q

cyclophosphamide indication

A

multisystem connective tissue disease or vasculitis e.g. Wegener’s, SLE

GvHD

85
Q

cyclophosphamide side effects

A

haemorrhagic cystitis + bladder cancer

haem malignancies

non-melanoma skin cancer

86
Q

methotrexate indication

A

autoimmune RA

psoriasis

crohn’s

anti-tumour

87
Q

methotrexate side effects

A

pneumonitis

pulmonary fibrosis

cirrhosis

88
Q

what is plasmaphoresis

A

remove pathogenic Ab from plasma

indicated in type 2 hypersensitivity reactions (goodpasture, myasthenia gravis, Ab-mediated rejection)

89
Q

what should you give with plasmaphoresis to prevent rebound Ab production

A

cyclophosphamide

90
Q

name some calcineurin inhibitors

A

tacrolimus

ciclosporin

91
Q

calcineurin inhibitor MOA

A

stop IL2 expression, blocking T cell proliferation

92
Q

calcineurin inhibitor indication

A

transplantation

93
Q

calcineurin inhibitor side effects

A

nephrotoxic

HTN

neurotoxic

diabetogenic

gum hypertrophy (ciclosporin)

94
Q

name an mTor inhibitor

A

sirolimus

95
Q

mTor inhibitor MOA

A

inhibit IL2 signaling pathway

96
Q

mTor inhibitor indication

A

transplantation

97
Q

mTor inhibitor side effects

A

HTN

less nephrotoxic than calcineurin inhibs

98
Q

name some JAK inhibitors

A

tofacitinib

ruxolitinib

99
Q

JAK inhibitor MOA

A

reduced production of inflammatory molecules

100
Q

JAK inhibitor indication

A

RA

101
Q

Name a PDE4 inhibitor

A

apremilast

102
Q

PDE4 inhibitor MOA

A

increase cAMP, reduce cytokine production

103
Q

PDE4 inhibitor indication

A

psoriasis

psoriatic arthritis

104
Q

basiliximab MOA

A

target IL-2R

prevent T cell proliferation

105
Q

natalizumab MOA

A

target alpha4 integrin

prevent T cell migration

106
Q

natalizumab indication

A

MS

107
Q

rabbit anti-thymocyte MOA

A

prevent T cell proliferation

108
Q

rituximab MOA

A

target CD20 on B cells, only epxressed on mature cells, not plasma cells

antobody level remains as plasma cell population in tact

deplete b cells for 6 months

109
Q

rituximab indication

A

B cell lymphomas

RA

SLE

110
Q

rituximab side effects

A

progressive multifocal leukoencephalopathy

111
Q

abatacept MOA

A

target CTLA4-Ig infused protein

enhance CTLA4 (which is an inhibitory checkpoint for T cell activation)

112
Q

abatacept indication

A

RA

113
Q

abatacept side effects

A

infection - TB, hep B/C

114
Q

which HLA antigens are expressed on all cells

A

HLA class 1 (A, B, C)

115
Q

which HLA antigens are expressed on antigen presenting cells

A

HLA class 2 (DR, DQ, DP)

116
Q

MHC class 1 present antigens to which class of T cells

A

CD8+

117
Q

MHC class 2 present antigens to which class of T cells

A

CD4+

118
Q

order of importance in HLA matching

A

DR > B > A

119
Q

hyperacute rejection pathology

A

preformed antibodies activate complement

thrombosis, necrosis

120
Q

hyperacute rejection treatment

A

prevent via HLA and ABO matching beforehand

121
Q

acute T cell mediated rejection pathology

A

type IV hypersensitivity, cell infiltration

122
Q

acute T cell mediated rejection treatment

A

T cell suppression - rabbit anti-thymocyte globulin or basiliximab

123
Q

acute antibody mediated rejection pathology

A

antibody attacks endothelium

vasculitis

124
Q

acute antibody mediated rejection treatment

A

rituximab (b cell suppression), Ab removal

125
Q

chronic rejection pathology

A

fibrosis, glomerulonephropathy, bronchiolitis obliterans

126
Q

chronic rejection treatment

A

prevent further organ damage

127
Q

GvHD symptoms

A

rash, N&V, abdo pain, diarrhoea, bloody stool, jaundice

128
Q

GvHD prophylaxis

A

cyclosporine, methotrexate, irradiate transfused blood products

129
Q

Disease associated with anti-CCP (cyclical citrullinated peptide)

A

RA

130
Q

Disease associated with anti-smooth muscle

A

autoimmune hepatitis

primary sclerosing cholangitis

131
Q

Disease associated with ant-Jo1

A

dermatomyositis

132
Q

dermatomyositis symptoms

A

heliotrope rash around eyes

gottron’s papules on dorsum of finger joint

difficulty climbing stairs, rising from chair

raised ESR, CK

133
Q

Disease associated with anti-centromere

A

limited systemic scleroderma

134
Q

limited systemic scleroderma symptoms

A

raynauds, oesphageal dysmotility, sclerodactyly, telangiectasia

raised ESR, hypergammaglobinaemia, reduced Hb

135
Q

Disease associated with anti-topoisomerase

A

diffuse systemic scleroderma

136
Q

diffuse systemic scleroderma symptoms

A

CREST + renal, cardiac and pulmonary involvement

137
Q

Disease associated with pANCA

A

churg-strauss syndrome

138
Q

churg-strauss symptoms

A

eosinophilia, asthma, chronic sinusitis, malaise, fatigue, weight loss

139
Q

Disease associated with anti-double stranded DNA

A

SLE

140
Q

Disease associated with anti-parietal cell

A

pernicious anaemia

141
Q

Disease associated with anti-Ro/ anti-La

A

sjogren’s syndrome (also SLE)

142
Q

Disease associated with anti-mitochondrial

A

primary biliary cirrhosis (pruritis, reduced absorption of soluble vitamins)

143
Q

Disease associated with anti-glutamic acid decarboxylase

A

T1DM

144
Q

Disease associated with cANCA

A

wegener’s granulomatosis

145
Q

wegener’s granulomatosis symptoms

A

epistaxis

saddle nose deformity

pulmonary haemorrhage

glomerulonephritis

treat with lifelong corticosteroid immunosuppression

146
Q

Disease associated with anti-endomysium, anti-ttg and anti-gliadin

A

coeliac

147
Q

Disease associated with anti-cardiolipin

A

antiphospholipid syndrome

SLE

syphilis

148
Q

Disease associated with anti-RNP

A

mixed connective tissue disease

149
Q

Disease associated with anti-nuclear

A

SLE, sjogren’s, autoimmune hepatitis, dermatopolymyositis

150
Q

Disease associated with anti-intrinsic factor

A

pernicious anaemia

151
Q

what does a high CH50 indicate

A

acute or chronic inflammation (ie RA)

152
Q

IgE roles

A

parasitic infections

also involved in mast cell activation in type 1 hypersensitivity

153
Q

coeliac HLA association

A

HLA DQ2

154
Q

ankylosing spondylitis HLA association

A

HLA B27

155
Q

goodpasture’s HLA association

A

HLA DR2

156
Q

HLA DR3 association

A

Graves

SLE

myasthenia gravis

157
Q

HLA DR4 association

A

T1DM

RA

158
Q

post-streptococcal rheumatic fever mechanism

A

molecular mimicry

159
Q

Di George’s mutation

A

22q11 deletion

160
Q

IFN-gamma receptor deficiency susceptiblity

A

intracellular pathogens (salmonella, TB)

161
Q

what other conditions is selective IgA deficiency linked to

A

autoimmune diseases (RA, SLE, coeliac)

162
Q

what mutation causes hyper IgM

A

mutation in CD40 ligand on T cells (X linked condition)

163
Q

what infection are hyper IgM patients susceptible to

A

pneumocystis jirovecci

164
Q

what is the mutation in common variable immunodeficiency

A

MHC class 3 –> aberrant class switching

increased risk of lymphomas, granulomas

clinical features - bronchiectasis, sinusitis

bloods - normal IgM, decreased IgA, IgG, IgE

165
Q

what Ig are type 1 hypersensitivity reactions mediated by

A

IgE

166
Q

gold standard for investigating type 1 hypersensitivity reactions

A

skin prick test

167
Q

what are serum tryptase levels useful for

A

diagnosing anaphylaxis

168
Q

what is the first immune component to initiate the response in ABO incompatibility

A

C1

169
Q

pemphigus vulgaris antibodies

A

anti-demoglein 1 and 3

170
Q

what can antibodies directed at chlamydia trachomatis cause

A

reactive arthritis (arthritis, dysuria, conjunctivitis, uveitis)

171
Q

which proteins are implicated in the pathogenesis of MS

A

proteolipid protein

myelin basic protein

172
Q

what antigens are the target for T cells in rheumatoid arthritis

A

synovial membrane antigens

173
Q

what is the name for a transplant from an identical twin

A

isograft

174
Q

what is an allograft

A

donor is same species as recipient but not identical

175
Q

what does ABO mismatch cause in transplantation

A

hyperacute rejection

176
Q

cyclosporine A MOA

A

calcineurin inhibitor

177
Q

azathioprine MOA

A

metabolised into 6-mercaptopurine - purine analogue that prevents DNA synthesis

178
Q

daclizumab target

A

CD25 of IL2 receptors - used in kidney transplant pts

179
Q

how does HIV bind to cell

A

gp120 binds to CXCR4 receptor on cd4 t cell

180
Q

IgG can be used to treat…

A

bruton’s agammaglobinaemia

hyper IgM syndrome

181
Q

haematopoietic stem cell transplant indications

A

SCID

leukaemia

MM

182
Q

interferon-alpha uses

A

Hep B

Hep C

Kaposi sarcoma

CML

183
Q

interferon-gamma uses

A

chronic granulomatous disease

184
Q

interferon-beta uses

A

MS

185
Q

severe mysthenic crisis management

A

plasmapheresis (also useful for goodpasture’s)

186
Q

what cytokine is adalimumab specific to

A

TNF-alpha

187
Q

infliximab MOA

A

TNF-alpha antagonist

188
Q

TNF-alpha antagonist uses

A

RA, crohn’s, ankylosing spondylitis

189
Q

denosumab MOA

A

antibody directed at RANK ligand –> inhibition of osteoclast function (prevent bone breakdown)

190
Q

denosumab uses

A

MM

osteoporosis

bone metastases

191
Q

dermatomyositis antibody

A

anti-jo1, anti-nuclear

192
Q

dermatomyositis clinical features

A

heliotrope rash around eyes

gottron’s papules on dorsum of finger joints

weakness of proximal limb muscles

193
Q

dermatomyositis immunofluoresence pattern

A

speckled

194
Q

peripheral pattern on immunofluoresence

A

anti-dsDNA (SLE)

195
Q

hashimoto’s thyroiditis antibodies

A

anti-thyroid peroxidase

anti-thyroglobulin

196
Q

autoimmune hepatitis type 1 antibodies

A

anti-nuclear antibodies

anti-smooth muscle antibodies

197
Q

autoimmune hepatitis type 2 (younger patients) antibodies

A

anti-liver kidney microsomal

198
Q

primary biliary cirrhosis antibodies

A

anti-mitochondrial

199
Q

autoantibodies to components of hemidesmosomes of the basement membrane

A

bullous pemphigoid

200
Q

autoantibodies to type 7 collagen

A

epidermolysis bullosa

201
Q

vitiligo antibodies

A

anti-melanocyte

202
Q

epistaxis, haemoptysis, positive for cANCA

A

wegener’s granulomatosis

203
Q

axial rigidity associated with a history of autoimmune disease and circulating anti-glutamic acid decarboxylase antibodies

A

stiff man syndrome

204
Q

target for autoantibodies in GBS

A

ganglioside LM1(A)

205
Q

assay for gold standard HIV diagnosis

A

western blot

206
Q

rapdily progressive glomerulonephritis with nosebleeds/ haemoptysis

A

wegener’s granulomatosis

207
Q

cd20

A

b cell

208
Q

what should new diagnoses of t1dm be screened for

A

coeliac

Path (hopefully more condensed) (5 decks)

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