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Pathology BB quizzes > Immunology > Flashcards

Immunology Flashcards

1
Q

what is Kostmann’s syndrome?

A

congenital neutropenia

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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

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3
Q

which drug class is most likely to induce acute angioedema?

A

ACE inhibitors (i.e. captopril)

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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

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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

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6
Q

management of angioedema with 2ary airway compromise

A

IM adrenaline 0.5 mL of 1:1000

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7
Q

what is the functional complement test used to investigate the classical pathway?

A

CH50

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8
Q

what is the functional complement test used to investigate the alternative pathway?

A

AP50

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9
Q

what complement factors are important chemotaxis agents?

A

C3a & C5a

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10
Q

what is the specific auto-antigen that is the target of the immune system in goodpasture?

A

type IV collagen

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11
Q

name the drug most likely to be used in the treatment of goodpasture?

A

prednisolone

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12
Q

what is affected in bare lymphocyte syndrome?

A

transcription factor proteins regulating expression of MHC class I or II

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13
Q

what is the immunoglobulin pattern seen in Wiskott-Aldrich syndrome?

A

decreased IgM and IgG

increased IgE and IgA

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14
Q

what are the features of Wiskott-Aldrich syndrome?

A

Thrombocytopenia, eczema, recurrent pyogenic infection

acronym: WATER

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15
Q

what mutations can lead to SCID?

A

IL-2R and ADA

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16
Q

what medical condition is a risk factor for developing chronic allograft rejection?

A

hypertension

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17
Q

what are the 3 important HLA subtypes to screen for in transplants in order of importance?

A

HLA DR > B > A

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18
Q

ABO incompatibility results in what type of transplant rejection?

A

hyperacute - preformed antibodies against a graft surface protein

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19
Q

what type of immunosuppressant results in a transient rise of white cells?

A

corticosteroids

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20
Q

which immunosuppressant gives gingival hypertrophy

A

ciclosporin

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21
Q

common side effects of ciclosporin

A

gingival hypertrophy

reduced GFR, hypertension and diabetes

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22
Q

common side effect of azathioprine

A

bone marrow supression

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23
Q

side effects of methotrexate?

A

pneumonitis, pulmonary fibrosis and cirrhosis

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24
Q

what blood test should be used to monitor MTX-induced cirrhosis?

A

procollagen III - NOT liver bioposy anymore

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25
Q

which is the Sabin vaccine?

is this inactivated or live attenuated?

A

oral polio

live attenuated

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26
Q

‘Extracts of or detoxified exotoxin product by a micro-organism’ is the vaccine for what?

A

tetanus

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27
Q

a vaccine made using recombinant DNA technology

A

Hepatitis B

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28
Q

The general name for a compound which increases the immune response to a vaccine without altering its specificity

A

adjuvant

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29
Q

A freeze dried live attenuated strain of Mycobacterium bovis

A

BCG

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30
Q

Agent used in humans that promotes a predominantly antibody response through the release of IL-4 that primes naïve B-cells.

A

Alum

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31
Q

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.

A

Polio, OPV, Sabin

..now give IPV. less immunogenic but more suitable for this population

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32
Q

first & second line treatment for RA?

A

NSAIDs

DMARD - MTX, SSZ, HCQ, leflonomide

…then…

TNFalpa inhibitor - adalimumab, infliximab, golimumab, certolizumab

rituximab (anti-CD20), abatercept (CD28 decoy), tocilizumab (anti-IL6 receptor)

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33
Q

prior to starting immunosuppression, what should be screened for and how?

A

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

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34
Q

what is deficient in bare lymphocyte syndrome?

A

MHC

type 1 = MHC I

type 2 = MHC II

(helpfully)

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35
Q

in BLS type 2, what is the immune profile you find on lab testing?

CD4, CD8, B cells, IgM, IgG

A

CD4 - low

CD8 - normal

B cells - normal

IgM - normal

IgG - low

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36
Q

bare lymphoctye syndrome type 2 is associated with what condition?

A

primary sclerosing cholangitis

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37
Q

what is the pathogenesis of BXL hypogammaglobulinaemia?

A

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

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38
Q

immunological replacement for patients with B cell disorders…

A

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

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39
Q

what are the common manifestations of early complement deficiency?

A

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
Q

what is antibacterial prophylaxis for asplenic patients who are pen-allergic?

A

erythromycin

41
Q

what is the management for Crohn’s disease?

A

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
Q

basiliximab

A

anti-CD25, used periop for tansplants to prevent rejection

inhibits T cells

43
Q

abatacept

A

decoy for APCs, resulting in less activation of T cells

used for anti-TNF resistant rheumatoid arthritis

44
Q

rituximab

A

anti-CD20 on B cells

depletes B cells and used in B cell lymphoma, SLE and rheumatoid arthritis

45
Q

natalizumab

A

binds to integrins, inhibits T cell migration

used for highly active relapsing remitting MS

46
Q

tocilizumab

A

anti-IL6 receptor, prevents proliferation of lymphoid and myeloid cells

used for RA and castleman’s disease

47
Q

what are the anti-TNF antibodies?

A

infliximab, golimumab, adalimumab, certolizumab

48
Q

etanercept

A

decoy TNF receptor

used in RA, ank spond, psoriasis, psoriatic arthritis

49
Q

ustekinumab

A

anti IL12/23, used to treat psoriasis

50
Q

denosumab

A

antibody against RANK-L, stops osteoclast differentiation

treatment of osteoporosis if cannot tolerate bisphosphonates

51
Q

muromonab-CD3/OKT3

A

first monoclonal ever

anti-CD3 used in treatment of acute transplant rejection

52
Q

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.

A

Kostmann syndrome

53
Q

immunosuppresiave agent that causes gum hyperplasia

A

cyclosporin

54
Q

what is the difference between sirolimus and tacrolimus?

A

sirolimus - targets T cell clonal proliferation

tacrolimus - targets T cell activation

55
Q

what medications and conditions are commonly associated with autoimmune haemolytic anaemia?

A

CLL and SLE

methyldopa and penicillin

56
Q

what infective agent is associated with the development of polyarteritis nodosa?

A

hepatitis B

57
Q

cyclophosphamide affects which lympocyte class more?

A

B cells > T cells

58
Q

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
A

cyclophosphamide

indicated for GPA, SLE, leukaemia and lymphoma

59
Q

what are the side effects of MMF?

A

bone marrow suppression

virus reactivation (herpes and JC virus)

60
Q

what is the Kveim test?

A

intradermal injection of spleen sample from sarcoid positive patient followed by biopsy looking for non-caseating granuloma formation 4-6 weeks later

61
Q

which complement investigation is different in a general inflammatory condition?

A

CH50 high indicates acute/chronic inflammation, as these are acute phase reactants

62
Q

what renal disease gives a low C3 but normal C4?

A

membranoproliferative GN (esp type III)

63
Q

what infections are you predisposed to if you have alternative complement pathway deficiency?

A

encapsulated bacteria

64
Q

neutrophil elastase gene (ELA2) is associated with what disease?

A

cyclic neutropenia

65
Q

what is an isograft?

A

transplant from a twin to another twin (or a donor that is genetically similar/identical)

66
Q

on immunoflourescence, what pattern do anti-Jo-1 antibodies display in dermatomyositis?

A

speckled

67
Q

which other nuclear antibodies display a speckled pattern on immunoflourescence?

A

anti-Jo-1 (DM/PM)

anti-Smith (SLE)

anti-Ro (Sjogren)

anti-RNP (MCTD)

68
Q

which nuclear antibodies display a homogeneous pattern on immunoflourescence?

A

anti-histone (drug induced SLE)

69
Q

which nuclear antibodies display a nucleolar pattern on immunoflourescence?

A

anti-RNA polymerase (systemic sclerosis)

70
Q

which nuclear antibodies display a peripheral pattern on immunoflourescence?

A

anti-dsDNA (SLE)

71
Q

what are the antibodies implicated in T1DM?

A

tyrosine phosphatase(s) - anti-IA2, anti-phogrin

islet cells - islet cell surface antibodies, islet cell antibodies (ICA)

enzymes - anti-GAD

insulin auto-antibodies

72
Q

male infant presents with ezcema, nail dystrophy and worsening alopecia universalis and bollous pemphigoid

treatment is BMT

A

IPEX syndrome

73
Q

what HLA subtype is most associated with coeliac? which is the 2nd most associated?

A

HLA DQ-2

DQ-8 is as well, but to a lesser extent

74
Q

which one is which between the AI blistering skin conditions?

A

pemphigus vulgaris - intercellulardesmoglein 3 in epidermis - NS positive

bullous pemphigoid - hemidesmosomes in basement membrane - NS negative

75
Q

what are the light microscopic findings to classify lupus nephritis from stage I - V?

A
  • 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
Q

what are the EM findings for classifying lupus nephritis from stages I - V?

A
  • I - mesangial immune deposits
  • II - mesangial immune deposits
  • III - subendothelial and mesangial immune deposits
  • IV - subendothelial immune deposits
  • V - subepithelial and intramembranous immune deposits
77
Q

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.

A

acute disseminated encephalomyelitis (ADEM)

78
Q

what is the antibody target in Guillain-Barre syndrome?

A

ganglioside LM1

79
Q

antibodies to ganglioside GM1 with motor nerve dysfunction

A

amyotrophic lateral sclerosis

80
Q

anti-Hu antibodies are associated with when?

A

paraneoplastic subacute sensory neuropathy (PSSN)

usually with a small cell lung CA underlying

81
Q

Western blot is commonly used as the diagnostic test for what condition?

A

HIV looking for anti-HIV antibodies

82
Q

HLA DR15/DR2

A

goodpastures

83
Q

HLA DR3

A

Type 1 DM, SLE and graves

84
Q

HLA DR4

A

type 1 DM and Rh arthritis

85
Q

what are 3 risk factors for chronic organ rejection?

A
  1. multiple acute organ rejections
  2. hypertension
  3. hyperlipidaemia
86
Q

what is the inheritance pattern for hyper IgM?

A

X linked

87
Q

what is the treatment for cyclic neutropenia?

what is the pattern of disease and remission?

A

G-CSF

occurs for 6 days every 3 weeks (roughly)

88
Q

a patient with a long standing inflammatory condition presents with miliary TB

what’s happened?

A

started on anti-TNF, iatrogenic

89
Q

what is the serum marker of antibody mediated transplant rejection?

A

C4d

90
Q

what is the difference in management between antibody and cellular mediated acute organ rejection?

A

antibody - plasmapheresis and B-cell immunosuppression (IVIG, rituximab)

cellular - t-cell immunosuppression (steroids, OKT/anti-thymocyte globulin)

91
Q

what is the treatment pre-/post-solid organ transplant?

A

pre: alemtuzumab (anti-CD52) or basiliximab (anti-CD25/IL-2R) or OKT/ATG
post: CNI + MMF/AZA +/- steroids

92
Q

what medication is used at GvHD prophylaxis in HSCT?

what is the treatment in an acute flare?

A

MTX + CNI (cyclosporine A or tacrolimus)

corticosteroids (topical if just skin disease, systemic if needed)

93
Q

when will an HIV test be falsely negative?

A

both screening (ELISA) and confirmatory (Western) test for antibodies, so patient must have seroconverted

first 10 weeks of infection (roughly) will be negative

94
Q

what cytokines are involved in the maturation and activation of eosinophils?

A

maturation - IL-3/5 & GM-CSF

activation - IgE and IL-4

95
Q

what are the antibodies produced against HIV by the adaptive immune system?

A

neutralising - anti-gp120 and anti-gp41

non-neutralising - anti-p24 gag IgG

96
Q

what was the vaccine that irradicated small pox?

A

Vaccinia

Pathology BB quizzes (5 decks)

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