Immunology Flashcards
1
Q
what is Kostmann’s syndrome?
A
congenital neutropenia
2
Q
24 y/o student develops worsening swelling of hands and geet and abdominal pain before exams. She has experienced similar episodes previously
what’s the diagnosis?
A
C1 inhibitor deficiency
3
Q
which drug class is most likely to induce acute angioedema?
A
ACE inhibitors (i.e. captopril)
4
Q
an intermittently itchy and desquamating skin rash in a particular distribution suggests…
A
contact hypersensitivity
c/o urticaria that does not result in skin break down
5
Q
drugs, dose and route for anaphylaxis
adult and paeds
A
adult
IM adrenaline 0.3 - 0.5 mg (1:1000 solution) q15 mins
1 - 2 L IV 0.9% NaCl at 5 - 10 mL/kg in first 5 mins
IV/IM diphenhydramine 25-50 mg & IV ranitidine 50 mg over 5 mins
IV methylprednisolone 1-2 mg/kg/day
peads
IM adrenaline 0.01 mg/kg (1:1000 solution) q15 mins, max 0.3 mg/dose
30 mL/kg IV 0.9% NaCl in first hour
IV/IM diphenhydramine 1-2 mg/kg & IV ranitidine 1 mg/kg over 5 mins
IV methylprednisone 1 - 2 mg/kg/day
6
Q
management of angioedema with 2ary airway compromise
A
IM adrenaline 0.5 mL of 1:1000
7
Q
what is the functional complement test used to investigate the classical pathway?
A
CH50
8
Q
what is the functional complement test used to investigate the alternative pathway?
A
AP50
9
Q
what complement factors are important chemotaxis agents?
A
C3a & C5a
10
Q
what is the specific auto-antigen that is the target of the immune system in goodpasture?
A
type IV collagen
11
Q
name the drug most likely to be used in the treatment of goodpasture?
A
prednisolone
12
Q
what is affected in bare lymphocyte syndrome?
A
transcription factor proteins regulating expression of MHC class I or II
13
Q
what is the immunoglobulin pattern seen in Wiskott-Aldrich syndrome?
A
decreased IgM and IgG
increased IgE and IgA
14
Q
what are the features of Wiskott-Aldrich syndrome?
A
Thrombocytopenia, eczema, recurrent pyogenic infection
acronym: WATER
15
Q
what mutations can lead to SCID?
A
IL-2R and ADA
16
Q
what medical condition is a risk factor for developing chronic allograft rejection?
A
hypertension
17
Q
what are the 3 important HLA subtypes to screen for in transplants in order of importance?
A
HLA DR > B > A
18
Q
ABO incompatibility results in what type of transplant rejection?
A
hyperacute - preformed antibodies against a graft surface protein
19
Q
what type of immunosuppressant results in a transient rise of white cells?
A
corticosteroids
20
Q
which immunosuppressant gives gingival hypertrophy
A
ciclosporin
21
Q
common side effects of ciclosporin
A
gingival hypertrophy
reduced GFR, hypertension and diabetes
22
Q
common side effect of azathioprine
A
bone marrow supression
23
Q
side effects of methotrexate?
A
pneumonitis, pulmonary fibrosis and cirrhosis
24
Q
what blood test should be used to monitor MTX-induced cirrhosis?
A
procollagen III - NOT liver bioposy anymore
25
which is the Sabin vaccine?
is this inactivated or live attenuated?
oral polio
live attenuated
26
'Extracts of or detoxified exotoxin product by a micro-organism' is the vaccine for what?
tetanus
27
a vaccine made using recombinant DNA technology
Hepatitis B
28
The general name for a compound which increases the immune response to a vaccine without altering its specificity
adjuvant
29
A freeze dried live attenuated strain of Mycobacterium bovis
BCG
30
Agent used in humans that promotes a predominantly antibody response through the release of IL-4 that primes naïve B-cells.
Alum
31
Live attenuated vaccine that is no longer given as standard in the UK since the rates of reverse mutation are higher than those of active disease.
Polio, OPV, Sabin
..now give IPV. less immunogenic but more suitable for this population
32
first & second line treatment for RA?
**NSAIDs**
**DMARD** - MTX, SSZ, HCQ, leflonomide
...then...
**TNFalpa inhibitor** - adalimumab, infliximab, golimumab, certolizumab
rituximab (anti-CD20), abatercept (CD28 decoy), tocilizumab (anti-IL6 receptor)
33
prior to starting immunosuppression, what should be screened for and how?
TB with CXR and TB ELISPOT
HBV and HCV - RNA and HBsAg
HIV
prior history of joint infection or implant (recurrence of septic arthritis or biofilm forming organism)
stop drug taking (snorting/injecting)
consider vaccinations
**malignancy** - prior CA diagnosis, advice on sunlight exposure
34
what is deficient in bare lymphocyte syndrome?
MHC
type 1 = MHC I
type 2 = MHC II
(helpfully)
35
in BLS type 2, what is the immune profile you find on lab testing?
CD4, CD8, B cells, IgM, IgG
CD4 - low
CD8 - normal
B cells - normal
IgM - normal
IgG - low
36
bare lymphoctye syndrome type 2 is associated with what condition?
primary sclerosing cholangitis
37
what is the pathogenesis of BXL hypogammaglobulinaemia?
*BTK* gene mutation, defect in B cell tyrosine kinase
Pre-B cells do not mature to B cells/plasma cells
No circulating Ig after 3 months old
Low IgM, IgG, IgA
38
immunological replacement for patients with B cell disorders...
All - pooled IgG given as infusion. administered every 3-4 weeks. aim to keep in normal ref range.
SCID and hyperIgM - bone marrow transplant (as these are T cell disorders really)
selective IgA - immunisations
39
what are the common manifestations of early complement deficiency?
immune complexes fail to activate immune system - fail to get cleared from plasma in circulation
results in immune complex deposition propensity
**SLE, kidney, joint and skin disease**
40
what is antibacterial prophylaxis for asplenic patients who are pen-allergic?
erythromycin
41
what is the management for Crohn's disease?
principle: always use whatever worked last time, then build from there
**1st line** - budesonide/5-ASAs to induce remission
**2nd line** - AZA/mercaptopurine + oral corticosteroid taper
**3rd line** - TNFalpha inhibitors + oral cortisosteroid taper
*n.b. if you use infliximab, must add AZA as well*
**maintenance** - thiopurines or MTX if intolerant
42
basiliximab
anti-CD25, used periop for tansplants to prevent rejection
inhibits T cells
43
abatacept
decoy for APCs, resulting in less activation of T cells
used for anti-TNF resistant rheumatoid arthritis
44
rituximab
anti-CD20 on B cells
depletes B cells and used in B cell lymphoma, SLE and rheumatoid arthritis
45
natalizumab
binds to integrins, inhibits T cell migration
used for highly active relapsing remitting MS
46
tocilizumab
anti-IL6 receptor, prevents proliferation of lymphoid and myeloid cells
used for RA and castleman's disease
47
what are the anti-TNF antibodies?
infliximab, golimumab, adalimumab, certolizumab
48
etanercept
decoy TNF receptor
used in RA, ank spond, psoriasis, psoriatic arthritis
49
ustekinumab
anti IL12/23, used to treat psoriasis
50
denosumab
antibody against RANK-L, stops osteoclast differentiation
treatment of osteoporosis if cannot tolerate bisphosphonates
51
muromonab-CD3/OKT3
first monoclonal ever
anti-CD3 used in treatment of acute transplant rejection
52
A 5-month-old boy is referred to a paediatrician after suffering with recurrent infections since his birth. His mother has noticed increased irritability. Blood tests reveal a neutrophil count of 350/mcL (N \>500/mcL). NBT test is normal.
Kostmann syndrome
53
immunosuppresiave agent that causes gum hyperplasia
cyclosporin
54
what is the difference between sirolimus and tacrolimus?
sirolimus - targets T cell clonal proliferation
tacrolimus - targets T cell activation
55
what medications and conditions are commonly associated with autoimmune haemolytic anaemia?
CLL and SLE
methyldopa and penicillin
56
what infective agent is associated with the development of polyarteritis nodosa?
hepatitis B
57
cyclophosphamide affects which lympocyte class more?
B cells \> T cells
58
A 46-year-old man with long-standing SLE is seen by his rheumatologist. He had previously been treated with corticosteroids, but has now developed end- organ involvement of his kidneys, lungs and heart.
What is the most appropriate drug choice?
* Cyclophosphamide
* Mycophenolate mofetil
* Basiliximab
* Abatacept
* Rituximab
* Efalizumab
* Infliximab
* Ustekinumab
* Denosumab
cyclophosphamide
indicated for GPA, SLE, leukaemia and lymphoma
59
what are the side effects of MMF?
bone marrow suppression
virus reactivation (herpes and JC virus)
60
what is the Kveim test?
intradermal injection of spleen sample from sarcoid positive patient followed by biopsy looking for non-caseating granuloma formation 4-6 weeks later
61
which complement investigation is different in a general inflammatory condition?
CH50 high indicates acute/chronic inflammation, as these are acute phase reactants
62
what renal disease gives a low C3 but normal C4?
membranoproliferative GN (esp type III)
63
what infections are you predisposed to if you have alternative complement pathway deficiency?
encapsulated bacteria
64
neutrophil elastase gene (ELA2) is associated with what disease?
cyclic neutropenia
65
what is an isograft?
transplant from a twin to another twin (or a donor that is genetically similar/identical)
66
on immunoflourescence, what pattern do anti-Jo-1 antibodies display in dermatomyositis?
speckled
67
which other nuclear antibodies display a speckled pattern on immunoflourescence?
anti-Jo-1 (DM/PM)
anti-Smith (SLE)
anti-Ro (Sjogren)
anti-RNP (MCTD)
68
which nuclear antibodies display a homogeneous pattern on immunoflourescence?
anti-histone (drug induced SLE)
69
which nuclear antibodies display a nucleolar pattern on immunoflourescence?
anti-RNA polymerase (systemic sclerosis)
70
which nuclear antibodies display a peripheral pattern on immunoflourescence?
anti-dsDNA (SLE)
71
what are the antibodies implicated in T1DM?
tyrosine phosphatase(s) - anti-IA2, anti-phogrin
islet cells - islet cell surface antibodies, islet cell antibodies (ICA)
enzymes - anti-GAD
insulin auto-antibodies
72
male infant presents with ezcema, nail dystrophy and worsening alopecia universalis and bollous pemphigoid
treatment is BMT
IPEX syndrome
73
what HLA subtype is **most** associated with coeliac? which is the 2nd most associated?
HLA DQ-2
DQ-8 is as well, but to a lesser extent
74
which one is which between the AI blistering skin conditions?
pemphigus vulgaris - intercellulardesmoglein 3 in epidermis - NS positive
bullous pemphigoid - hemidesmosomes in basement membrane - NS negative
75
what are the light microscopic findings to classify lupus nephritis from stage I - V?
* I - nothing
* II - changes only to the mesangium
* III - focal, segmental or GN affecting \<50% glomeruli
* IV - focal, segmental or GN involving \>50% glomeruli
* V - glomerular sclerosis involving \>90% glomeruli, fibrosis and tubular atrophy
76
what are the EM findings for classifying lupus nephritis from stages I - V?
* I - mesangial immune deposits
* II - mesangial immune deposits
* III - subendothelial and mesangial immune deposits
* IV - subendothelial immune deposits
* V - sub*epithelial* and intramembranous immune deposits
77
a demyelinating condition that follows vaccination or infection. Clinical features include fever, headache and reduced consciousness; focal signs include optic neuritis, cranial nerve palsies and seizures. Most cases are followed by recovery within a few months.
acute disseminated encephalomyelitis (ADEM)
78
what is the antibody target in Guillain-Barre syndrome?
ganglioside LM1
79
antibodies to ganglioside GM1 with motor nerve dysfunction
amyotrophic lateral sclerosis
80
anti-Hu antibodies are associated with when?
paraneoplastic subacute sensory neuropathy (PSSN)
usually with a small cell lung CA underlying
81
Western blot is commonly used as the diagnostic test for what condition?
HIV looking for anti-HIV antibodies
82
HLA DR15/DR2
goodpastures
83
HLA DR3
Type 1 DM, SLE and graves
84
HLA DR4
type 1 DM and Rh arthritis
85
what are 3 risk factors for chronic organ rejection?
1. multiple acute organ rejections
2. hypertension
3. hyperlipidaemia
86
what is the inheritance pattern for hyper IgM?
X linked
87
what is the treatment for cyclic neutropenia?
what is the pattern of disease and remission?
G-CSF
occurs for 6 days every 3 weeks (roughly)
88
a patient with a long standing inflammatory condition presents with miliary TB
what's happened?
started on anti-TNF, iatrogenic
89
what is the serum marker of antibody mediated transplant rejection?
C4d
90
what is the difference in management between antibody and cellular mediated acute organ rejection?
antibody - plasmapheresis and B-cell immunosuppression (IVIG, rituximab)
cellular - t-cell immunosuppression (steroids, OKT/anti-thymocyte globulin)
91
what is the treatment pre-/post-solid organ transplant?
pre: alemtuzumab (anti-CD52) or basiliximab (anti-CD25/IL-2R) or OKT/ATG
post: CNI + MMF/AZA +/- steroids
92
what medication is used at GvHD prophylaxis in HSCT?
what is the treatment in an acute flare?
MTX + CNI (cyclosporine A or tacrolimus)
corticosteroids (topical if just skin disease, systemic if needed)
93
when will an HIV test be falsely negative?
both screening (ELISA) and confirmatory (Western) test for antibodies, so patient must have seroconverted
first 10 weeks of infection (roughly) will be negative
94
what cytokines are involved in the maturation and activation of eosinophils?
maturation - IL-3/5 & GM-CSF
activation - IgE and IL-4
95
what are the antibodies produced against HIV by the adaptive immune system?
neutralising - anti-gp120 and anti-gp41
non-neutralising - anti-p24 gag IgG
96
what was the vaccine that irradicated small pox?
Vaccinia
