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
2
Q
child with recurrent bacterial infections. blood count shows neutropenia. NBT test is normal
A
Kostmann syndrome
3
Q
autosomal dominant condition with neutropenia every 3 weeks or so
A
cyclic neutropenia
4
Q
condition with a very high neutrophil count, but absent pus formation. NBT test positive. Delayed umbilical cord sloughing
A
leukocyte adhesion deficiency
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
6
Q
patient with diarrhoea, fever, tiredness. chest infections since diarrhoea, Crohn’s history
A
protein-losing enteropathy
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
8
Q
Elevated IgM, no IgG, IgA or IgE
A
Hyper IgM syndrome
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
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
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
12
Q
Sclerosing cholangitis with hepatomegaly and jaundice. MHC 1 deficiency –> CD8 T cell deficiency
A
Type 1 bare lymphocyte syndrome
13
Q
Sclerosing cholangitis with hepatomegaly and jaundice. MHC 2 deficiency –> CD4 T cell deficiency
A
Type 2 bare lymphocyte syndrome
14
Q
Recurrent mild resp/ GI infections, anaphylaxis to transfusions
A
Selective IgA deficiency
15
Q
Sickle cell anaemia patients are vulnerable to which type of infection
A
Encapsulated bacteria (S. pneumonia)
16
Q
Autosomal recessive condition resulting in severe sepsis a few days after birth. Absolute deficiency in both granulocytes and lymphocytes
A
Reticular dysgenesis
17
Q
ADA deficiency cell count abnormalities
A
Low B, T and NK cells
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
19
Q
Most important HLA antigens to match
A
HLA-A, HLA-B, HLA-DR
20
Q
Name a monogenic auto-inflammatory disease
A
Familial mediterranean fever
21
Q
Pathogens you are susceptible to in chronic granulomatous disease
A
catalase positive (pseudomonas, listeria, aspergillus, candida, E. coli, s. aureus, serratia)
22
Q
What do all patients with C2 deficiency have
A
SLE
severe skin disease, increased infections
23
Q
Patients with increased susceptibility to infection in MBL deficiency
A
premature infants, chemotherapy, HIV, Ab deficiency
24
Q
alternative complement pathway deficiencies lead to increased infections with…
A
encapsulated bacteria
25
SLE pathogenesis
production of immune complexes leading to consumption of C3 and C4
26
Ix for classical pathway activity
CH50
27
Ix for alternative pathway activity
AP50
28
factors of classical complement pathway
C1q, C1r, C1s, C2, C4
29
most common classical pathway deficiency
C2
30
common pathway factors
C3, C5-9
31
deficiency of common pathway factors leads to infections with...
N. meningitidis
S. pneumoniae
H. influenzae
32
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
ADA deficiency
33
What protects SCID infant for first 3 months
maternal IgG
34
autoinflammatory conditions are a defect in which type of immunity
innate
35
autoimmune conditions are a defect in which type of immunity
adaptive
36
familial mediterranean fever symptoms
periodic fevers and serositis
37
familial mediterranean fever pathogenesis
MEFV mutation --> abnormal TNFa/ IL1 signalling
38
familial mediterranean fever treatment
colchicine
anakinra (IL1-R antagonist)
etanercept (TNFa inhibitor)
39
name a polygenic autoinflammatory disease
crohn's
40
name a mixed pattern disease
Ankylosing spondylitis
psoriatic arthritis
behcet's
41
mixed pattern disease pathogenesis
HLA-B27 mutation affects CD8 T cells, Th17 cells, NK cells
42
name a polygenic autoimmune disease
rheumatoid arthritis, myasthenia gravis, pernicious anaemia, SLE
43
Goodpasture disease HLA association
HLA-DR15
44
Grave's disease HLA association
HLA-DR3
45
SLE HLA association
HLA-DR3
46
T1DM HLA association
HLA-DR3/4
47
Rheumatoid arthritis HLA association
HLA-DR4
48
Name a monogenic autoimmune disease
APECED
IPEX
ALPS
49
APECED features
mutation of AIRE transcription factor means lack of negative T cell selection
autoimmune polyendocrinopathy candidiasis ectodermal dystrophy
50
IPEX features
mutation in FoxP3 required fpr T reg cell development
Treg cells CD25+, FoxP3x
immune dysregulation, polyendocrinopathy, enteropathy, X-linked
diarrhoea, diabetes, dermatitis
51
ALPS features
mutation in Fas pathway --> T cells not killed in thymus
large spleen + lymph nodes, autoimmune cytopenia, lymphoma
autoimmune lymphoproliferative syndrome
52
type 1 hypersensitivity mechanism
sensitisation in primary exposure
Ag cross links IgE on mast cells --> degranulation
53
type 1 hypersensitivity example
atopic eczema
anaphylaxis
54
type 2 hypersensitivity mechanism
antibody binds to surface antigen
55
type 2 hypersensitivity example
Graves, myasthenia gravis
56
type 3 hypersensitivity mechanism
antibody-antigen complexes deposit in tissue
57
type 3 hypersensitivity example
SLE, serum sickness
58
type 4 hypersensitivity mechanism
T cell mediated
CD4+ release cytokines
CD8+ kill target cells
59
type 4 hypersensitivity example
T1DM, contact dermatitis
60
live attenuated vaccines examples
MMR (HIV safe)
Varicella zoster
BCG (NOT HIV safe)
Yellow fever (NOT HIV safe)
oral polio, typhoid
61
inactivated vaccine example
polio (salk)
anthrax
influenza
hep a
62
component/ subunit vaccine example
Hep B
HPV
Influenza
63
Toxoid vaccine example
diptheria
tetanus
pertussis
64
conjugate vaccine example
N. meninigitidis
Haemophilus influenza B
Strep pneumoniae
65
DNA/ mRNA vaccine example
covid vaccines
66
human normal immunoglobulin uses
ITP, Kawasaki, GBS, measles
67
what is pavilizumab
monoclonal antibody for RSV
68
interferon alpha uses
Hep B, C and CML
Kaposi's sarcoma
hairy cell leukaemia
multiple myeloma
69
interferon beta uses
behcet's
relapsing MS
70
interferon gamma use
chronic granulomatous disease
71
which antibody is responsible for protecting against GI infections
IgA
72
ipilimumab receptor and indication
CTLA4
melanoma
73
moromonab receptor and indication
CD3
transplant rejection
74
dacilizumab receptor and indication
IL2
transplant rejection
75
denosumab receptor and indication
RANKL
osteoporosis, bone mets pain, hypercalcaemia
76
infliximab receptor and indication
TNF-alpha
IBD
77
how do corticosteroids cause immunosuppression
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
78
azathioprine MOA
stops DNA production (metabolised to purine analogue 6-mercaptopurine)
affects T cells more than B
79
azathioprine indication
transplantation
autoimmune and autoinflammatory diseases
80
mycophenolate MOA
blocks de novo guanosine synthesis
affects T cells more than B cells
81
mycophenolate indication
transplantation
autoimmune and vasculitis
82
mycophenolate side effects
HSV reactivation
JC virus reactivation --> progressive multifocal leukoencephalopathy (PML)
progressive neural demyelination condition
83
cyclophosphamide MOA
alkylates gaunine base --> stops DNA replication
affects B cells more than T cells
84
cyclophosphamide indication
multisystem connective tissue disease or vasculitis e.g. Wegener's, SLE
GvHD
85
cyclophosphamide side effects
haemorrhagic cystitis + bladder cancer
haem malignancies
non-melanoma skin cancer
86
methotrexate indication
autoimmune RA
psoriasis
crohn's
anti-tumour
87
methotrexate side effects
pneumonitis
pulmonary fibrosis
cirrhosis
88
what is plasmaphoresis
remove pathogenic Ab from plasma
indicated in type 2 hypersensitivity reactions (goodpasture, myasthenia gravis, Ab-mediated rejection)
89
what should you give with plasmaphoresis to prevent rebound Ab production
cyclophosphamide
90
name some calcineurin inhibitors
tacrolimus
ciclosporin
91
calcineurin inhibitor MOA
stop IL2 expression, blocking T cell proliferation
92
calcineurin inhibitor indication
transplantation
93
calcineurin inhibitor side effects
nephrotoxic
HTN
neurotoxic
diabetogenic
gum hypertrophy (ciclosporin)
94
name an mTor inhibitor
sirolimus
95
mTor inhibitor MOA
inhibit IL2 signaling pathway
96
mTor inhibitor indication
transplantation
97
mTor inhibitor side effects
HTN
less nephrotoxic than calcineurin inhibs
98
name some JAK inhibitors
tofacitinib
ruxolitinib
99
JAK inhibitor MOA
reduced production of inflammatory molecules
100
JAK inhibitor indication
RA
101
Name a PDE4 inhibitor
apremilast
102
PDE4 inhibitor MOA
increase cAMP, reduce cytokine production
103
PDE4 inhibitor indication
psoriasis
psoriatic arthritis
104
basiliximab MOA
target IL-2R
prevent T cell proliferation
105
natalizumab MOA
target alpha4 integrin
prevent T cell migration
106
natalizumab indication
MS
107
rabbit anti-thymocyte MOA
prevent T cell proliferation
108
rituximab MOA
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
rituximab indication
B cell lymphomas
RA
SLE
110
rituximab side effects
progressive multifocal leukoencephalopathy
111
abatacept MOA
target CTLA4-Ig infused protein
enhance CTLA4 (which is an inhibitory checkpoint for T cell activation)
112
abatacept indication
RA
113
abatacept side effects
infection - TB, hep B/C
114
which HLA antigens are expressed on all cells
HLA class 1 (A, B, C)
115
which HLA antigens are expressed on antigen presenting cells
HLA class 2 (DR, DQ, DP)
116
MHC class 1 present antigens to which class of T cells
CD8+
117
MHC class 2 present antigens to which class of T cells
CD4+
118
order of importance in HLA matching
DR > B > A
119
hyperacute rejection pathology
preformed antibodies activate complement
thrombosis, necrosis
120
hyperacute rejection treatment
prevent via HLA and ABO matching beforehand
121
acute T cell mediated rejection pathology
type IV hypersensitivity, cell infiltration
122
acute T cell mediated rejection treatment
T cell suppression - rabbit anti-thymocyte globulin or basiliximab
123
acute antibody mediated rejection pathology
antibody attacks endothelium
vasculitis
124
acute antibody mediated rejection treatment
rituximab (b cell suppression), Ab removal
125
chronic rejection pathology
fibrosis, glomerulonephropathy, bronchiolitis obliterans
126
chronic rejection treatment
prevent further organ damage
127
GvHD symptoms
rash, N&V, abdo pain, diarrhoea, bloody stool, jaundice
128
GvHD prophylaxis
cyclosporine, methotrexate, irradiate transfused blood products
129
Disease associated with anti-CCP (cyclical citrullinated peptide)
RA
130
Disease associated with anti-smooth muscle
autoimmune hepatitis
primary sclerosing cholangitis
131
Disease associated with ant-Jo1
dermatomyositis
132
dermatomyositis symptoms
heliotrope rash around eyes
gottron's papules on dorsum of finger joint
difficulty climbing stairs, rising from chair
raised ESR, CK
133
Disease associated with anti-centromere
limited systemic scleroderma
134
limited systemic scleroderma symptoms
raynauds, oesphageal dysmotility, sclerodactyly, telangiectasia
raised ESR, hypergammaglobinaemia, reduced Hb
135
Disease associated with anti-topoisomerase
diffuse systemic scleroderma
136
diffuse systemic scleroderma symptoms
CREST + renal, cardiac and pulmonary involvement
137
Disease associated with pANCA
churg-strauss syndrome
138
churg-strauss symptoms
eosinophilia, asthma, chronic sinusitis, malaise, fatigue, weight loss
139
Disease associated with anti-double stranded DNA
SLE
140
Disease associated with anti-parietal cell
pernicious anaemia
141
Disease associated with anti-Ro/ anti-La
sjogren's syndrome (also SLE)
142
Disease associated with anti-mitochondrial
primary biliary cirrhosis (pruritis, reduced absorption of soluble vitamins)
143
Disease associated with anti-glutamic acid decarboxylase
T1DM
144
Disease associated with cANCA
wegener's granulomatosis
145
wegener's granulomatosis symptoms
epistaxis
saddle nose deformity
pulmonary haemorrhage
glomerulonephritis
treat with lifelong corticosteroid immunosuppression
146
Disease associated with anti-endomysium, anti-ttg and anti-gliadin
coeliac
147
Disease associated with anti-cardiolipin
antiphospholipid syndrome
SLE
syphilis
148
Disease associated with anti-RNP
mixed connective tissue disease
149
Disease associated with anti-nuclear
SLE, sjogren's, autoimmune hepatitis, dermatopolymyositis
150
Disease associated with anti-intrinsic factor
pernicious anaemia
151
what does a high CH50 indicate
acute or chronic inflammation (ie RA)
152
IgE roles
parasitic infections
also involved in mast cell activation in type 1 hypersensitivity
153
coeliac HLA association
HLA DQ2
154
ankylosing spondylitis HLA association
HLA B27
155
goodpasture's HLA association
HLA DR2
156
HLA DR3 association
Graves
SLE
myasthenia gravis
157
HLA DR4 association
T1DM
RA
158
post-streptococcal rheumatic fever mechanism
molecular mimicry
159
Di George's mutation
22q11 deletion
160
IFN-gamma receptor deficiency susceptiblity
intracellular pathogens (salmonella, TB)
161
what other conditions is selective IgA deficiency linked to
autoimmune diseases (RA, SLE, coeliac)
162
what mutation causes hyper IgM
mutation in CD40 ligand on T cells (X linked condition)
163
what infection are hyper IgM patients susceptible to
pneumocystis jirovecci
164
what is the mutation in common variable immunodeficiency
MHC class 3 --> aberrant class switching
increased risk of lymphomas, granulomas
clinical features - bronchiectasis, sinusitis
bloods - normal IgM, decreased IgA, IgG, IgE
165
what Ig are type 1 hypersensitivity reactions mediated by
IgE
166
gold standard for investigating type 1 hypersensitivity reactions
skin prick test
167
what are serum tryptase levels useful for
diagnosing anaphylaxis
168
what is the first immune component to initiate the response in ABO incompatibility
C1
169
pemphigus vulgaris antibodies
anti-demoglein 1 and 3
170
what can antibodies directed at chlamydia trachomatis cause
reactive arthritis (arthritis, dysuria, conjunctivitis, uveitis)
171
which proteins are implicated in the pathogenesis of MS
proteolipid protein
myelin basic protein
172
what antigens are the target for T cells in rheumatoid arthritis
synovial membrane antigens
173
what is the name for a transplant from an identical twin
isograft
174
what is an allograft
donor is same species as recipient but not identical
175
what does ABO mismatch cause in transplantation
hyperacute rejection
176
cyclosporine A MOA
calcineurin inhibitor
177
azathioprine MOA
metabolised into 6-mercaptopurine - purine analogue that prevents DNA synthesis
178
daclizumab target
CD25 of IL2 receptors - used in kidney transplant pts
179
how does HIV bind to cell
gp120 binds to CXCR4 receptor on cd4 t cell
180
IgG can be used to treat...
bruton's agammaglobinaemia
hyper IgM syndrome
181
haematopoietic stem cell transplant indications
SCID
leukaemia
MM
182
interferon-alpha uses
Hep B
Hep C
Kaposi sarcoma
CML
183
interferon-gamma uses
chronic granulomatous disease
184
interferon-beta uses
MS
185
severe mysthenic crisis management
plasmapheresis (also useful for goodpasture's)
186
what cytokine is adalimumab specific to
TNF-alpha
187
infliximab MOA
TNF-alpha antagonist
188
TNF-alpha antagonist uses
RA, crohn's, ankylosing spondylitis
189
denosumab MOA
antibody directed at RANK ligand --> inhibition of osteoclast function (prevent bone breakdown)
190
denosumab uses
MM
osteoporosis
bone metastases
191
dermatomyositis antibody
anti-jo1, anti-nuclear
192
dermatomyositis clinical features
heliotrope rash around eyes
gottron's papules on dorsum of finger joints
weakness of proximal limb muscles
193
dermatomyositis immunofluoresence pattern
speckled
194
peripheral pattern on immunofluoresence
anti-dsDNA (SLE)
195
hashimoto's thyroiditis antibodies
anti-thyroid peroxidase
anti-thyroglobulin
196
autoimmune hepatitis type 1 antibodies
anti-nuclear antibodies
anti-smooth muscle antibodies
197
autoimmune hepatitis type 2 (younger patients) antibodies
anti-liver kidney microsomal
198
primary biliary cirrhosis antibodies
anti-mitochondrial
199
autoantibodies to components of hemidesmosomes of the basement membrane
bullous pemphigoid
200
autoantibodies to type 7 collagen
epidermolysis bullosa
201
vitiligo antibodies
anti-melanocyte
202
epistaxis, haemoptysis, positive for cANCA
wegener's granulomatosis
203
axial rigidity associated with a history of autoimmune disease and circulating anti-glutamic acid decarboxylase antibodies
stiff man syndrome
204
target for autoantibodies in GBS
ganglioside LM1(A)
205
assay for gold standard HIV diagnosis
western blot
206
rapdily progressive glomerulonephritis with nosebleeds/ haemoptysis
wegener's granulomatosis
207
cd20
b cell
208
what should new diagnoses of t1dm be screened for
coeliac
