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immuno Flashcards

1
Q

what is mutated in reticular dysgenesis (autosomal recessive severe SCID)

A

adenylate kinase 2 (AK2)

  • a mitochondrial enzyme metabolism enzyme
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2
Q

what is mutated in Kostmann syndrome

A

HAX-1 (HCLS1 associated protein X-1)

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3
Q

severe congenital neutropenia with HAX1 mutation

A

Kostmann syndrome

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4
Q

episodic neutropenia every 4-6 weeks, mutation in ELA2

A

cyclic neutropenia

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5
Q

what is mutated in cyclic neutropenia

A

neutrophil elastase ELA-2

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6
Q

what is the inheritance of cyclic neutropenia

A

autosomal dominant

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7
Q

mutation in AK2 + low neutrophils, lymphocytes, macrophages, platelets, requiring bone marrow transplantation

A

reticular dysgenesis

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8
Q

what is part of the adaptive immune response?

A

B lymphocytes - producing antibodies
T lymphocytes
cytokines and chemokines

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9
Q

what is part of the innate immune response?

A

neutrophils, basophils, eosinophils
macrophages & monocytes
NK cells
Dendritic Cells

Acute phase proteins
complement
cytokines and chemokines

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10
Q

recurrent infections, very high neutrophil counts in blood during infection, absence of pus formation, delayed umbilical cord separation.

A

leukocyte adhesion deficiency

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11
Q

in leukocyte adhesion deficiency, what is the mutation/ deficiency?

A

CD18 deficiency.

CD11a/DC18 and CD11b/CD18 expressed on neutrophils regulate neutrophil adhesion and transmigration

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12
Q

what receptors are involved in neutrophil adhesion and transmigration?

A

CD11a/ CD18

CD11b/CD18

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13
Q

what complement proteins are involved in the classically pathway?

A

C1, C2, C4

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14
Q

what is the main complement protein that is activated by the classical, alternative and lectin pathway?

A

C3

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15
Q

Nitroblue Tetrazolium test abnormal/ negative

A

chronic granulomatous disease

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16
Q

in chronic granulomatous disease, what is deficient?

A

NADPH oxidase

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17
Q

Which complement proteins are involved in the lectin pathway?

A

C2 and C4

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18
Q

which complement protein is involved in the alternative pathway?

A

C3

C3 binds directly to
techoic acid on Gram + bacteria and to LPS on gram - bacteria

+ involves factors B, I, P

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19
Q

mx of chronic granulomatous disease

A

IFN gamma

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20
Q

dihydrorhodamine flow cytometry test abnormal / negative

A

chronic granulomatous disease

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21
Q

chronic granulomatous disease pathology

A

absent respiratory burst due to deficiency of one of the components of NADPH oxidase.

impaired killing if intracellular microorganisms and excessive inflammation with granuloma formation

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22
Q

chronic granulomatous disease presentation

A

susceptibility to bacteria esp catalase + bacteria.

granuloma formation + lymphadenopathy + hepatosplenomegaly

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23
Q

susceptibility to infection with mycobacteria (TB and atypical), BCG, Salmonella. What condition?

A

deficiency of IFNgamma/ IL12 and their receptors.

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24
Q

what happens when one is deficient in early classical pathway? ie. C1/2/4

A

immune complexes fail to activate complement pathway -> increased susceptibility to infection

increased load of self-antigens

deposition of immune complexes which stimulated inflammation in skin, joints, kidneys e.g. SLE

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25
Q

SLE associated with which complement deficiencies?

A

most common C2

C1q, C1r, C1s, C2 and C4 deficiency all seen in SLE

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26
Q

complement deficient - susceptibility to?

A

encapsulated bacteria

e.g. neisseria meningitidis,
strep pneumoniae, h influenza

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27
Q

active lupus ix?

A

Complement levels
first low C4
then low C4 and low C3

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28
Q

increased infection in patients who have another cause of immune impairment e.g. premature infant, HIV infection, chemotherapy, antibody deficiency

A

MBL deficiency

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29
Q

low T cells and NK cells, normal B cells, but low Igs.

A

X linked SCID

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30
Q

4 month old, infections of all types, failure to thrive, persistent diarrhoea

A

SCID

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31
Q

what immune cells are vital for protection against viral infections and tumours?

A

NK cells and CD8 T cells

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32
Q

DiGeorge’s syndrome

A

CATCH 22

Cardiac abnormalities (esp Tetralogy of Fallot)
Abnormal facies (low set ears, high forehead)
Thymic aplasia
Cleft palate
HypoCa/ HypoPTH

Chr22- deletion at 22q11.2

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33
Q

Thymic aplasia - reduced numbers of T cells. which condition?

A

DiGeorge’s

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34
Q

Absent expression of MHC Class II molecules

A

Bare Lymphocyte Syndrome type II

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35
Q

absent expression of MHC Class I molecules

A

Bare lymphocyte syndrome type I

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36
Q

Bare lymphocyte Syndrome type II

A 
absent MHC Class II
-> profound deficiency of CD4+ T cells
\+ failure to produce IgA/ IgG because no class switching

infections of all types.

may be associated with sclerosing cholangitis
FTT after 3 months

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37
Q

Hyper IgM syndrome aka

A

CD40L deficiency

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38
Q

what gene is defective in Bruton’s X linked hypogammaglobulinaemia

A

B cell tyrosine kinase gene

BTK gene

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39
Q

absence of mature B cells and no circulating Ig. Recurrent infections. Family history where brother is affected.

A

X linked Bruton’s hypogammaglobulinaemia

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40
Q

recurrent respiratory tract and GI tract infections

A

selective IgA deficiency

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41
Q

Selective IgA deficiency patients are at risk of what during blood transfusion?

A

anaphylaxis due to introduction of donor IgA

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42
Q

High IgM, undetectable Ig G, Ig A, Ig E. Normal number circulating B cells.

failure of class switching due to T cell defect.

recurrent infections- e.g. h influenza, strep pneumo, pseudomonas, pneumocystis jiroveci

A

Hyper IgM syndrome

aka CD40L defciency

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43
Q

what gene is mutated in Hyper IgM syndrome?

A

CD40 Ligand gene

CD40L normally on T cell

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44
Q

Other than HIV, what other condition increases risk of pneumocystis jiroveci infection?

A

Hyper IgM syndrome

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45
Q

what are the likely pathogens in antibody/ B cell or CD4 T cell deficiency?

A 
Strep pneumo
H influenzae
Strep pyogenes
pseudomonas
\+ tetanus/ diphtheria + enterovirus
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46
Q

no germinal centre development within lymph nodes and spleen. No class switching occurring.

A

Hyper IgM syndrome

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47
Q

histology shows spongiosis + inflammatory infiltrate in the dermis + dilated dermal capillaries. if chronic: crusting, scaling, lichenification.

A

Atopic dermatitis/ contact dermatitis

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48
Q

defect in which protein predisposes patients with atopic dermatitis to staph aureus superinfection?

A

B defensin

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49
Q

exposure to birch pollen induces allergy to stony fruits (due to cross reactivity) e.g. melons, rosacea fruit. symptoms usually limited to mouth e.g. swollen tongue, lips

A

oral allergy syndrome

tx: avoid food. if ingested, wash mouth and take antihistamine.
2% get anaphylaxis and require adrenaline

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50
Q

latex allergy + allergy to banana/ papaya/ potato/ avocado/ kiwi/ mango/ melon

A

latex food syndrome

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51
Q

nasal itch and runny nose/ congestion, sneezing, anosmia. On examination: pale bluish swollen nasal mucosa

A

allergic rhinitis

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52
Q

ix of allergic rhinitis

A

skin prick test

or RAST for specific IgE

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53
Q

mx of allergic rhinitis

A

oral antihistamine or

steroid nasal spray

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54
Q

what differentiates acute from chronic urticaria?

A

acute: wheals which resolve completely within 6 weeks.

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55
Q

mx of acute urticaria

A

antihistamines

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56
Q

mx of anaphylaxis

A

1: IM 1:1000 adrenaline

ABC + seek help
Oxygen, elevate legs, inhaled bronchodilators, IV hydrocortisone (to prevent late phase response), chlorphenamine, IV fluids,

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57
Q

Gold standard test for food and drug allergy

A

double blind oral food challenge
- increasing volumes of offending food/ drug ingested under close supervision
but risk of severe reaction when testing

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58
Q

after an acute anaphylactic episode, how to confirm via ix?

A

measure serum mast cell tryptase (should return to baseline by 6h)

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59
Q

first line test for allergy

A

skin prick (more sensitive and specific than blood tests)

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60
Q

what is type II hypersensitivity?

A

Antibody mediated hypersensitivity.

antibody specific to cells/ organs

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61
Q

haemolytic disease of the newborn: what antibodies against what antigen? How to diagnose?

A

anti-D antibodies IgG -> rhesus antigens on neonatal erythrocytes.

diagnosis via positive direct antiglobulin Coombs test

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62
Q

autoimmune haemolytic anaemia

what antibodies against what antigen? How to diagnose?

A

autoantibodies against antigens on RBC surface. -> destruction of RBCs

low Hb, high reticulocytes, high Br, high LDH

Diagnosis by positive direct anti globulin Coombs Test

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63
Q

haemolytic disease of the newborn tx?

A

maternal plasma exchange, exchange transfusion of neonate

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64
Q

Autoimmune haemolytic anaemia treatment

A

steroids

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65
Q

autoimmune thrombocytopenic purpura
what antibodies to what antigens?

what type of hypersensitivity?

A

anti platelet antibody to gpIIb/IIIa on platelets

Type II hypersensitivity

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66
Q

autoimmune thrombocytopenic purpura features?

A

bruising/ bleeding/ purpura

low platelets

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67
Q

goodpastures syndrome

what type hypersensitivity? what antibody to what antigen?

A

Type II hypersensitivity

Anti-GBM ab to type IV collagen on basement membrane found in kidneys and lung

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68
Q

Diagnosis of Goodpastures?

A

anti GBM Ab

linear smooth IF staining of IgG deposits on BM
-> crescentic (rapid progressive glomerulonephritis on BM)

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69
Q

Goodpastures Syndrome Tx?

A

corticosteroids and immunosuppression

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70
Q

pemphigus vulgaris

what type of hypersensitivity?
what antibody against what antigen?

A

type II hypersensitivity

anti-desmoglein 3 antibody to epidermal cadherin

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71
Q

anti-desmoglein 3 antibody

to epidermal cadherin

A

pemphigus vulgaris

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72
Q

Graves Disease

what antibody to what antigen?
what type of hypersensitivity?

A

anti-TSHR antibody to TSH R

type II hypersensitivity

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73
Q

Graves Disease tx

A

carbimazole and propylthiouracil

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74
Q

Myasthenia gravis
what antibody to what antigen?
what type hypersensitivity?

A

anti-AchR ab to AchR

type II hypersensitivity

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75
Q

fatiguable muscle weakness, double vision, ptosis, tensilon test +ve, abnormal EMG

A

myasthenia gravis

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76
Q

myasthenia gravis tx

A

anticholinesterase (neostigmine, pyridostigmine)

if severe: e.g. affecting breathing. Plasmapheresis and IVIG

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77
Q

pernicious anaemia

what type hypersensitivity? what antibody to what antigen?

A

anti-IF and anti-parietal cells Ab to IF and parietal cells.

type II hypersensitivity

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78
Q

churg-Strauss syndrome (eosinophilic granulomatosis with polyangitis)

what antibodies to what antigen? what type hypersensitivity?

A

p-ANCA binds to medium and small vessels.

type II hypersensitivity

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79
Q

hypereosinophilia, asthma + vasculitis.

p-ANCA +ve

A

Churg-Strauss

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80
Q

wegener’s granulomatosis (granulomatosis with polyangitis)

what antibodies to what antigen?
what type hypersensitivity?

A

c-ANCA binds to medium and small vessels -> vasculitis

type II hypersensitivity

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81
Q

Wegeners, Churg-strauss, microscopic polyangitis tx?

A

cyclophosphamide

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82
Q

microscopic polyangiitis.

what antibody to what antigen? what type hypersensitivity?

A

p-ANCA to small vessels -> vasculitis

type II hypersensitivity

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83
Q

persistent itchy wheals lasting >6 wks + angiooedema in response to triggers e.g. medications, cold, exercise, food

A

chronic urticaria

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84
Q

urticarial eruption, often painful + bruising/ post inflammatory residual pigmentation at site of itching

A

urticarial vasculitis

inflammation of small blood vessels

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85
Q

chronic urticaria mx

A

avoid precipitants,

preventative antihistamine.

Im adrenaline for pharyngeal angioedema

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86
Q

what is type III hypersensitivity?

A

immune complex mediated - deposition of immune complexes in joints/ kidneys etc

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87
Q

SLE

what type hypersensitivity?

A

type III hypersensitivity. immune complex deposition in joints, kidneys,

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88
Q

cryoglobulins causing joint pain, splenomegaly, skin, nerve and kidney involvement. sometimes associated with hep C infection.

A

Essential mixed cryoglobulinaemia

Type III hypersensitivity

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89
Q

serum sickness

what antibody to what antigen? what type hypersensitivity?

A

type III hypersensitivity

antibodies react to antiserum (e.g. antivenom / drugs like penicillin) forming Immune complexes that deposit.

gives rise to rashes, itching, arthralgia, lymphadenopathy.

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90
Q

what drugs may caused drug induced SLE?

A

hydralazine, procainamide, isoniazid

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91
Q

SLE Ix/ serum results?

A

reduced C4, may have reduced C3 if severe disease
antibodies to ds-DNA, ANA (Ro/La/ Smith), histones

ESR high, usually normal CRP

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92
Q

polyarteritis nodosa

what type of hypersensitivity?

A

type III

immune complex deposition in vessels -> aneurysm development

fever, arthralgia, skin, nerve, kidney involvement. pericarditis and MI.

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93
Q

what is type IV hypersensitivity?

A

T cell mediated hypersensitivity. delayed hypersensitivity.

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94
Q

TIDM

what type hypersensitivity?
What antibodies to what antigens?

A

type IV hypersensitivity

anti glutamate decarboxylase antibodies (GAD converts Glutamate to GABA which stimulates insulin release)
anti- islet cell antibodies to pancreatic islet cells
anti insulin antibodies to insulin
anti-IA2 antibodies to tyrosine phosphotase

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95
Q

CSF shows oligoclonal bands of IgG on electrophoresis

A

Multiple sclerosis

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96
Q

multiple sclerosis

what type hypersensitivity?
what antibodies to what antigen?

A

type IV hypersensitivity (Th1 cells)

antibodies to myelin basic protein, proteolipid protein

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97
Q

rheumatoid arthritis

what type hypersensitivity
what antibodies to what antigens?

A

type IV hypersensitivity

anti-CCP
rheumatoid factor (anti-IgG igM)
to antigens in synovial membrane

also type III. due to immune complexes formed

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98
Q

most specific antibody for rheumatoid arthritis

A

anti-CCP ab

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99
Q

what are some environmental risk factors of rheumatoid arthritis?

A

smoking

gum infection with porphyromonas gingivalis

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100
Q

mx of rheumatoid arthritis

A

analgesia e.g. NSAIDs
steroids
1. DMARDS e.g. methotrexate
2. TNFa e.g. infliximab, etanercept, adalimuimab

anti-IL6 e.g. tocilizumab
anti-CTLA4 Ig e.g. abatacept
JAK inhibitor e.g. tofacitinib

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101
Q

what are some complications of rheumatoid arthritis?

A

lung fibrosis, rheumatoid nodules, anaemia of chronic disease

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102
Q

mx of crohns disease

A

anti inflammatory drugs
e.g. mesalazine
TNFa antagonists e.g. infliximab
steroids

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103
Q

what HLA is associated with goodpastures syndrome?

A

HLA DR15/ DR2

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104
Q

HLA DR3 assoc with which diseases?

A

Graves disease
SLE
T1DM

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105
Q

HLA DR 4 assoc w?

A

rheumatoid arthritis

T1DM

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106
Q

anti centromere antibodies

A

limited cutaneous scleroderma (CREST syndrome)

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107
Q

other than antitopoisomerase, what other antibodies are found in diffuse cutaneous scleroderma?

A

antibodies to RNA polymerase I, II, III

fibrillarin antibodies

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108
Q

anti Ro and anti La antibodies

A

sjogrens syndrome

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109
Q

xerostermia, keratoconjunctivis sic. schirmers +ve. parotid/ salivary gland enlargement.

A

sjogrens syndrome

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110
Q

what is the most persistent autoantibody, even after tx? in coeliac disease?

A

anti-gliadin antibody

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111
Q

IPEX syndrome- what is it?

A

immune dysregulation, polyendocrinopathy, enteropathy and X-linked inheritance + autoimmune diseases

the only cure is bone marrow transplant.

eczematous dermatitis, nail dystrophy, autoimmune skin conditions such as alopecia universalis and bullous pemphigoid

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112
Q

immune dysregulation, polyendocrinopathy, enteropathy and X-linked inheritance + autoimmune diseases
what condition?

A

IPEX syndrome

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113
Q

anti cardiolipin and anti lupus anticoagulant antibodies

A

antiphospholipid syndrome

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114
Q

antiphospholipid syndrome antibodies?

A

anti cardiolipin
anti B2 glycoprotein
anti lupus anticoagulant

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115
Q

autoimmune hepatitis antibodies?

A

anti smooth muscle
anti LKM1 (liver-kidney-microsomal 1)
anti soluble liver antigen (SLA)

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116
Q

autoimmune hepatitis in paediatric population w poor response to steroid. which autoantibody?

A

anti LKM1 (liver kidney microsomal 1)

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117
Q

anti smooth muscle
anti LKM1 (liver-kidney-microsomal 1)
anti soluble liver antigen (SLA)

what condition

A

autoimmune hepatitis

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118
Q

churg-strauss syndrome antibody?

A

p-ANCA to myeloperoxidase

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119
Q

congenital heart block in infants of mothers w SLE. what antibody?

A

anti-Ro antibody

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120
Q

dermatitis herpetiformis. what antibody?

A

anti-endomysial IgA antibody

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121
Q

dermatomyositis what antibody?

A

anti-Jo 1 (to tRNA synthetase)

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122
Q

hashimotos thyroiditis what antibodies?

A

antibodies to thyroid peroxidase and thyroglobulin

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123
Q

mixed connective tissue disease

antibody?

A

anti-U1RNP antibody (speckled pattern)

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124
Q

ANAs?

A

anti ro / la/ smith/ u1RNP

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125
Q

what is the most sensitive specific in pernicious anaemia?

A

anti parietal cells antibody

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126
Q

complications of pernicious anaemia?

A

peripheral neuropathy

subacute combined degeneration of the cord

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127
Q

what proportion of sjogrens patients have +ve Rheumatoid factor?

A

60-70%

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128
Q

IFN gamma therapy for?

A

chronic granulomatous disease

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129
Q

IFN alpha therapy for?

A

Hep B, Hep C
Kaposis sarcome
hairy cell leukaemia, chronic myelogenous leukaemia, malignant myeloma

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130
Q

IFN beta therapy for?

A

relapsing Multiple sclerosis

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131
Q

pembrolizumab

A

anti-PD1 antibody

allows T cell activation. indications: advanced melanoma, lung cancer

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132
Q

ipilimumab

A

antibody for CTLA4 on T cell -

allows T cell activation

advanced melanoma

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133
Q

advanced melanoma immunological tx?

A

ipilimumab (anti CTLA4) and pembrolizumab (anti PD1) on T cells to activate T cells

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134
Q

BMT indications?

A

secondary to haem malignancy

SCID/ leukocyte adhesion defect

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135
Q

what is the receptor binding and membrane fusion glycoprotein of influenza virus?

A

haemagglutinin

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136
Q

what are the anti proliferative agents? (cytotoxic agents that inhibit DNA synthesis)

A

Mycophenolate Mofetil

Azathioprine

Cyclophosphamide

Methotrexate

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137
Q

antiproliferative agent that inhibits de novo purine (e.g. adenine, guanine) synthesis and preferentially inhibits T cell activation and proliferation?

A

Azathioprine

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138
Q

antiproliferative agent that inhibits guanine synthesis and preferentially inhibits T over B cell proliferation?

A

mycophenolate mofetil

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139
Q

antiproliferative agent that inhibits dihydrofolate reductase therefore decreasing DNA synthesis (anti folate)

A

methotrexate

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140
Q

antiproliferative agent that alkylates guanine base of the DNA and affects B cells over T cells, but at high doses affects all cells w high turnover

A

cyclophosphamide

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141
Q

immunosuppressant that causes transient neutrophilia

A

prednisolone

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142
Q

immunosuppressant that increases risk of osteoporosis

A

prednisolone

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143
Q

immunosuppressant that works by reducing prostaglandin synthesis and may increase risk of cushings

A

prednisolone

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144
Q

antiproliferative agent against connective tissue disease e.g. SLE, vasculitis e.g. Wegener’s,

+ SEs of increased risk of bladder ca, haemorrhage cystitis, infection/ bone marrow suppression

A

cyclophosphamide

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145
Q

antiproliferative agent used in transplantation, autoimmune diseases, vasculitis and has SEs of infection especially herpes virus reactivation and progressive multifocal leukoencephalopathy

A

Mycophenolate mofetil

146
Q

Progressive multifocal leukoencephalopathy what virus?

A

JC virus

147
Q

antiproliferative agent used in transplantation, autoimmune diseases and auto inflammatory disease such as crohns and Ulcerative colitis

+ if ppl have TPMT polymorphism, are unable to metabolise the drug

A

Azathioprine

148
Q

antiproliferative agent that is used in RA, psoriasis, Crohns, and in chemo. + SEs such as folate deficiency causing megaloblastic macrocytic anaemia + pneumonitis/ pulm fibrosis and hepatotoxicity

A

methotrexate

149
Q

calcineurin inhibitors?

A

tacrolimus and cyclosporin

calcineurin normally activates IL-2. -> calcineurin inhibitors reduce T cell activation and proliferation

150
Q

calcineurin inhibitor with side effects of nephrotoxicity, neurotoxicity, HTN, dysmorphic features and gum hypertrophy

A

cyclosporin

151
Q

calcineurin inhibitor with side effects of nephrotoxicity, neurotoxicity, HTN and is diabetogenic

A

tacrolimus

152
Q

anti CD25 / anti IL2R (alpha chain)

A

Basiliximab

153
Q

what are calcineurin inhibitors indicated in?

A

transplant rejection prophylaxis by reducing T cell activation and proliferation

154
Q

what is Sirolimus? and some side effects?

A

Sirolimus blocks clonal proliferation of T cells, is used for transplant rejection prophylaxis and has fewer side effects than tacrolimus. SEs are hypertension and a little nephrotoxicity

155
Q

what is tofacitinib?

A

a JAK inhibitor used in rheumatoid arthritis

156
Q

What is apremilast?

A

a PDE4 inhibitor used in psoriasis and psoriatic arthritis

157
Q

anti CD25 antibody, inhibits T cell proliferation, used pre and post surgery to prevent allograft rejection

A

Basilixumab

158
Q

anti CTLA4-Ig, reduces T cell activation, may be used in Rheumatoid arthritis

A

Abatacept

159
Q

anti-CD20, depletes mature B cells

A

Rituximab

160
Q

Rituximab indicated for?

A

anti-CD20, depletes mature B cells.

lymphoma, rheumatoid arthritis, SLE

161
Q

anti-alpha4 integrin (binds to VCAM1 to mediate rolling/arrest of leukocytes)

A

Natalizumab

162
Q

Natalizumab - what is is used for

A

an anti-alpha4 integrin antibody used inhibit T cell migration
-> relapsing and remitting MS, crohns disease

163
Q

anti-IL6 R antibody

A

Tocilizumab

164
Q

Tocilizumab indicated for?

A

anti-IL6 R antibody
reduces macrophage, T cell, B Cell, neutrophil activation

used in Rheumatoid arthritis, castleman’s disease

165
Q

mouse monoclonal antibody against OKT3, blocks CD3 on T cells

A

muromonab-CD3

166
Q

muromonab used for?

A

blocks CD3 on t cells

indicated in active allograft transplant rejection (as T cells are the main mediator of acute organ rejection)

167
Q

anti RANK Ligand antibody

A

denosumab

168
Q

indications for denosumab?

A

osteoporosis, multiple myeloma, bone mets

anti-RANK ligand antibody -> reduces osteoclast differentiation and function

169
Q

anti-IL12/23

A

ustekinumab.

170
Q

ustekinumab indicated for?

A

psoriasis/ psoriatic arthritis

171
Q

anti-IL17A

A

secukinumab

172
Q

secukinumab indicated for?

A

psoriasis, psoriatic arthritis, ankylosing spondylitis

173
Q

anti-TNFa antibodies

A

infliximab, adalimumab (fully human monoclonal ab), certolizumab, golimumab

for rheumatic arthritis, ankylosing spondylitis, psoriasis, psoriasis arthritis, inflammatory bowel disease

174
Q

TNF alpha antagonist

A

etanercept

175
Q

anti-thymocyte globulin indicated for?

A

lymphocyte depletion. for allograft rejection (renal/ heart)

176
Q

monoclonal antibody that binds to CD52 found on lymphocytes resulting in depletion

A

alemtuzumab

for CLL, MS

177
Q

IL2 receptor antibody, targets CD25, for organ transplant rejection prophylaxis (not Basiliximab)

A

Daclizumab

178
Q

3 stages of transplant rejection

A

recognition of foreign antigen

activation of antigen specific lymphocytes

effector function (T cells etc)

179
Q

where are HLA A / B / C molecules expressed

A

MHC class I expressed on all cells

180
Q

where are HLA DR/DQ/DP molecules expressed

A

MHC Class II expressed on antigen presenting cells but can also be unregulated on other cells under stress.

181
Q

what antigens are important in allograft transplant?

A

HLA- MHC on chr6
ABO blood antigens
Minor HLA

182
Q

Acute transplant rejection is usually due to what sort of recognition?

A

direct presentation

whereby DONOR APC present antigen and/ MHC to recipient T cells

183
Q

chronic transplant rejection is usually due to what sort of recognition?

A

indirect presentation

whereby Recipient APC presents donor antigen to recipient T cells.

184
Q

what are the different effectors in the effector phase of graft rejection?

A
  1. graft infiltration by alloreactive CD4 cells
  2. Cytotoxic cell death - release of toxins to kill target, punch holes in target cells, apoptotic cell death
  3. macrophages- phagocytosis
  4. antibodies bind to graft endothelium
185
Q

hyper acute transplant rejection

time frame?
mechanism?

A

min- hours

preformed antibodies in recipient activate complement

186
Q

hyper acute transplant rejection

time frame?
mechanism?
pathology?
treatment?

A

time frame: min- hours

mechanism: preformed antibdoies which activate complement

prevention via cross match (ABO) and HLA matching

pathology shows thrombosis and necrosis

187
Q

actue (cellular) transplant rejection

time frame?
mechanism?
pathology?
treatment?

A

time frame: weeks to months

mechanism: CD4 activating a type IV reaction
pathology: cellular infiltrate, interstitial inflammation and tubulitis
treatment: T cell immunosuppression (e.g. calcinuerin inhibitors)

188
Q

acute (antibody mediated) transplant rejection

time frame?
mechanism?
pathology?
treatment?

A

time frame: weeks- months

mechanism: B cells producing antibodies that attack vessels in donor organ/tissue
pathology: vasculitis, C4d
treatment: antibody removal and B cell immunosuppression

189
Q

chronic transplant rejection

time frame?
mechanism?
pathology?
treatment?

A

time frame: months to years

mechanism: immune and non-immune mechanism. multiple acute rejections, hypertension, hyperlipidaemia, multiple infections
pathology: fibrosis, glomerulopathy (kidneys), vasculopathy (ischaemia), bronchiolitis obliterans (lungs)
treatment: minimise organ damage

190
Q

Graft vs Host Disease

time frame?
mechanism?
pathology?
treatment?

A

time frame: days to weeks

mechanism: donor lymphocytes attacking host tissue
pathology: skin (rash), gut (diarrhoea, vomiting, bloody stool), and liver (jaundice) involvement

Treatment: prevention/ immunosuppression- corticosteroids

191
Q

Acute vascular rejection

when does this occur?

A

after xenograft

similar to hyperacute but 4-6 days after transplant

192
Q

before a transplant what should you always check in donor and recipient?

A

determine donor and recipient blood group and HLA type and maximise similarity

Check recipients pre formed antibodies against ABO and HLA

Cross match between donor and recipient. (tests if serum from recipient is able to bind/ kill donor lymphocytes)

193
Q

after transplant, what should you do to check if patient will or will not reject graft?

A

After transplant: check again for new antibodies vs the graft (via cytotoxicity assay/ flow cytometry/ solid phase assay)

194
Q

before a transplant, what medication would you give to induce the patient?

A

suppress T cell response

e.g. anti-CD25 Basiliximub or anti-CD52 Alemtuzumab or OKT3 (muromonab)/ Anti-thymocyte globulin

195
Q

after a transplant, what medication would you give to the patient to reduce rejection?

A

immunosuppressants

e.g. calcineurin inhibitors + mycophenolate mofetil/ azathioprine +/- steroids

196
Q

how to treat episodes of acute rejection. medications against cellular rejection?

A

steroids. OKT3/ anti-thymocyte globulin

197
Q

how to treat episodes of acute rejection. medications against antibody-mediated rejection?

A

IVIG, plasma exchange, anti-CD20 (e.g. rituximab), anti-C5

198
Q

what happens in a haematopoietic stem cell transplant

A

eliminate host immune system via total body irradiation/ cyclophosphamide/ etc

replace with own (autologous) or HLA matched donor (allogeneic) bone marrow

199
Q

what is graft vs host disease?

A

occurs in allogeneic haemotopoietic stem cell transplan where donor lymphocytes react against host tissues.

related to level of HLA incompatibility

200
Q

GvHD prophylaxis?

A

methotrexate

cyclosporine

201
Q

symptoms of GVHD?

A

skin (rash)
GI (diarrhoea, vomiting, abdo pain, nausea, bloody stools)
liver (jaundice)

202
Q

GvHD - treatment?

A

corticosteroids

203
Q

complications post transplantation?

A

increased risk of infections (bacterial, viral, fungal)

+ opportunistic infections due to immunosuppression (e.g. BK virus, CMV, pneumocystis carinii)

+ malignancy risk

  • viral associated (x100) e.g. Kaposi’s sarcoma, lymphoproliferative disease (EBV)
  • skin cancer (x20)
  • other cancers e.g. lung, colon (x2-3)

+ atherosclerosis
- Hypertension, hyperlipidaemia (x20) increase risk of death from MI

204
Q

HIV virus targets?

A

CD4+ T helper cells

also CD4+ monocytes and dendritic cells

205
Q

What viral proteins are used in HIV infection?

A

reverse transcriptase to convert HIV RNA into DNA

integrase which integrates HIV DNA into host cells genes

Protease that allows for cleavage of proteins into final virus after viral transcription and protein synthesis

206
Q

how does HIV virus bind to host cell?

A

binds via gp120 on CD4+ T cells

then conformational change via gp41 for entry into cell

207
Q

HIV co-receptors?

A

CXCR4 / CCR5 on macrophages

208
Q

what are the neutralising antibodies of the HIV virus?

A

anti-gp120

anti-gp41

209
Q

what are the non-neutralising antibodies of the HIV virus?

A

anti-p24 gag IgG

the gag protein is an intrastructural support for HIV

210
Q

what are the naturally produced chemokines that prevent HIV entry?

A

MIP-1a, MIP-1b and RANTES

produced by CD8 T cells
-these block co-receptors.

211
Q

what is the median time from infection with HIV to development of AIDS in typical progressors?

A

8-10 years

212
Q

what is the median time from infection with HIV to development of AIDS in rapid progressors?

A

2-3 years

213
Q

what is the median time from infection with HIV to development of AIDS in long term non progressors?

A

stable CD4 counts and no symptoms even after 10 years

214
Q

what factor predicts disease progression?

A

initial viral burden

215
Q

what is the screening test for HIV?

A

antibody test for anti-HIV ab via ELISA

usually done from 1 month onwards and if v high risk, again at 2 months

216
Q

what is the confirmation test for HIV?

A

usually done when the screening test is positive.

detect Ab via western blot
or
PCR for HIV RNA

217
Q

what has happened in the patient for HIV screening test to be +ve

A

Patient must have seroconverted.

seroconversion is the period of time during which HIV antibodies develop and become detectable.
Seroconversion occurs usually 1-3 wks after infection, but could take up to 6 months.
often not always accompanied by flu like symptoms including fever, rash, swollen lymph nodes and muscle aches.

218
Q

After diagnosis of HIV, what do you want to monitor in the patient?

A 
Viral Load (via PCR)
CD4 Count (via FACS)

AIDS = <200 cells/ microL

219
Q

HIV patient Tx?

A

commence combination antiretroviral therapy immediately once diagnosis of HIV is confirmed

usually 2NRTIs + Protease inhibitor/ NNRTI or 2nd line: integrase inhibitor

this allows substantial control of viral replication, increase in cd4 t cell counts, and improvement in their host defences

220
Q

if HIV+ mother has viral load <50, would you recommend breastfeeding her child?

A

still recommend formula feeding the child

221
Q

in Pregnancy, what can you do reduce HIV transmission from mother to child?

A
  1. take antiretroviral therapy and aim Viral load <50
  2. avoid breastfeeding after birth and choose formula feeding
  3. have a C-section if VL>50 and recommended by specialist team
222
Q

what treatment should babies born to HIV+ mothers get after birth?

A

baby should be given antiretrovirals (3 drug combination) within 4 h and continued until 4-6 wks of age if VL> 50

zidovudine monotherapy if VL<50

223
Q

what tests will baby born to HIV+ mother need after birth?

A

tested for HIV during first 2 days of life, on discharge from hospital, at 6 wks and at 12 wks. A further test at 18 months old.

224
Q

If baby is tested positive for HIV, what important treatment?

A

PCP prophylaxis with co-trimoxazole

225
Q

if untreated HIV+ve woman presents in labour , what tx?

A

stat dose of nevirapine, and commence fixed dose zidovudine with lamivudine and raltegravir.

IV zidovudine infused for the duration of labour and delivery

226
Q

if untreated HIV+ve woman is pregnant, what tx to start?

A

combivir (zidovudine + lamivudine)
truvada (tenofovir + emtricitabine)
kivexa (abacavir + lamivudine)

227
Q

what is maraviroc?

A

an HIV drug. CCR5 antagonist, attachment inhibitor

228
Q

what is enfuvirtide?

A

a HIV fusion inhibitor

229
Q

some e.g.s of nukes (nucleoside RTIs) used in HIV

A

zidovudine, abacavir, emtricitabine, lamivudine

230
Q

tenofovir

A

a nucleotide RTI

231
Q

efavirenz?

A

a non nucleoside RTI

SEs include CNS effects

232
Q

dolutegravir, raltegravir?

A

integrase inhibitors.

233
Q

what is contraindicated in individuals with confirmed anaphylactic hypersensitivity to egg products?

A

influenza

234
Q

e.g.s of live attenuated vaccines

A

a Yellow Chicken TOMB

Yellow Fever
Chicken Pox
Typhoid
Oral Polio
MMR
BCG
235
Q

e.g.s of component/ subunit vaccines

A

Hep B (HbS Ag)
HPV (Capsid)
Influenza (haemagluttinin, neuraminadase)

236
Q

e.g. of inactivated toxoid vaccine

A

diphtheria

tetanus

237
Q

e.g. of conjugate vaccines

A

PMH
pneumococcus
meningococcus
Hib

238
Q

e.g. of inactivated vaccines

A

HIP CRAB

Hep A
Inactivated Polio
Pertussis

Cholera
Rabies
Anthrax
Bubonic Plague

239
Q

what do adjuvants do?

A

increase the immune response without altering its specificity

240
Q

what adjuvant is a water-in-oil emulsion containing mycobacterial cell wall components? not used clinically in humans

A

complete Freund’s adjuvants

241
Q

what adjuvant is an unmethylated DNA motif, which activates TLR-9 on APCs, thus stimulating expression of costimulatory molecules?

A

CpG

242
Q

what adjuvant helps adsorb antigens and then releases the antigens slowly and also induces mild inflammatory reaction, thus increasing the development of the adaptive immune response?

A

aluminium salts

e.g. in Hep A, B, Hib vaccines

243
Q

what adjuvant is a multimeric antigen with adjuvant built in?

A

ISCOMs

immune stimulating complex

244
Q

what is pavilizumab?

A

a monoclonal antibody for RSV

245
Q

a monogenic anti-inflammatory disease where patient gets periodic fevers lasting 48-96h assoc w abdo pain (peritonitis), chest pain (pleurisy and pericarditis), arthritis. Defect in Pyrin-marenostrin, coded in MEFV gene.

A

familial Mediterranean fever

an auto recessive condition

lack of pyrin-marenostrin means excessive TNFa, IL1 (inflammatory cytokines) production

246
Q

what sites are not normally exposed to the immune system?

A

eyes

testes

CNS

247
Q

a monogenic autoimmune disease, with mutation in AIRE (an autoimmune regulator)
- antibodies against parathyroid and adrenal glands -> Addison’s and hypoPTH
and antibodies agains IL17 & IL22 (predisposing to candidiasis)

A

Autoimmune polyendocrine syndrome type 1 (APS-1)

aka

APECED syndrome

248
Q

a monogenic autoimmune disease with a mutation in the FAS pathway -> failure of apoptosis of lymphocytes. features of increased lymphocytes + Lymphadenopathy and splenomegaly and is assoc w lymphoma.

A

ALPS

autoimmune lymphoproliferative syndrome

249
Q

a monogenic autoimmune disease with mutation in Foxp3 (required for development of Treg cells) -> endocrinopathy (T1DM, thyroid), diarrhoea, eczematous dermatitis.

A

IPEX syndrome

250
Q

contraindications to skin prick testing.

A

extensive eczema, dermatographism, previous anaphylactic reaction, antihistamines should be discontinued 48h before testing.

251
Q

lung cancer due to heavy smoking habit. what mutation is associated with the subtype of lung cancer pt is most likely to develop.

A

p53 and RB1 - small cell carcinoma
p53, c-myc, kras- squamous cell carcinoma

p53

252
Q

put the viruses susceptible to acyclovir in order of susceptibility

A

herpes simplex 1 > herpes simplex 2 > varicella zoster

253
Q

fever, conjunctivitis, maculopapular rash starting on face + spreading to whole body. + cervical and occipital lymphadenopathy

A

Rubella

  • sub occipital and post auricular lymphadenopathy
254
Q

infiltrative heart diseases like amyloidosis, haemochromatosis and sarcoidosis.

what type of cardiomyopathy are pts with these conditions likely to develop?

A

restrictive cardiomyopathy.

there is impaired ventricular compliance, and patient have big atria albeit a normal heart size.

255
Q

what is the earliest visible gross lesion in atherosclerosis?

A

fatty streak

256
Q

asbestos exposure -> greatest risk of which kind of lung cancer?

A

bronchial carcinoma.

although the strongest risk factor for mesothelioma is asbestos exposure.

257
Q

v low leucocyte alkaline phosphate + neutrophilia

A

chronic myeloid leukaemia.
the low leukocyte alkaline phosphatase is a result of low activity in malignant neutrophils.

if accompanied by immature erythrocytes, then it will be termed a leukoerythroblastic reaction, caused by fibrosis or infiltration of the BM.

258
Q

used in treatment of highly active relapsing-remitting multiple sclerosis and crohns disease

A

natalizumab.

binds to a4-integrin found on VCAM1 to arrest leucocyte migration.

259
Q

what immune therapies increase risk of progressive multifocal leucoencephalopathy due to activation of the JC virus?

A

mycophenolate mofetil, rituximab, natalizumab.

any neuropsychiatric changes in patients on treatment should prompt further investigation.

260
Q

triad of neurological abnormality, respiratory compromise after recent hx of total hip replacement surgery.

A

fat embolism

261
Q

swollen gums, brusiing, petechiae, anaemia, poor wound healing, peifollicular and subperiosteal haemorrhages, corkscrew hair, haemarthrosis

A

vitamin C deficiency.

Scurvy.

262
Q

what protein in synthesised more by the liver in response to inflammatory cytokines in anaemia of chronic disease?

A

hepcidin.

the liver produces more hepcidin in response to IL-6. Hepcidin reduces iron absorption by the small intestines and causes iron accumulation in macrophages. together, these processes cause anaemia.

263
Q

histology of someone after a myocardial infarction shows normal histology, CK-MB is also normal. how long has it been since the coronary event happened?

A

under 6 hours.

264
Q

histology of someone after a myocardial infarction shows granulation tissue, new blood vessels, myofibroblasts, collagen synthesis. how long has it been since the coronary event happened?

A

1-2 wks

265
Q

histology of someone after a myocardial infarction shows a strengthening, decellularising scar. how long has it been since the coronary event happened?

A

wks - months

266
Q

histology of someone after a myocardial infarction shows infiltration of polymorphs but debris still present. how long has it been since the coronary event happened?

A

1-4 days

267
Q

histology of someone after a myocardial infarction shows removal of debris. how long has it been since the coronary event happened?

A

5-10 days

268
Q

histology of someone after a myocardial infarction shows loss of nuclei, homogenous cytoplasm, necrotic cell death. how long has it been since the coronary event happened?

A

6- 24 hours

269
Q

what do you see in MEN1?

A

parathyroid hyperplasia/ adneoma, pituitary adenoma, pancreatic endocrine tumour (often pheochromocytoma)

270
Q

what do you see in MEN2A?

A

parathyroid, medullary thyroid cancer, phaeochromocytoma.

271
Q

what do you see in MEN 2B?

A

medullary thyroid cancer, phaeochoromocytoma, neuroma, marfanoid phenotype.

272
Q

what organism responsible for infective endocarditis in a patient undergoing colonoscopy?

A

streptococcus bovis

273
Q

mTOR inhibitor?

A

sirolimus

274
Q

magnesium deficiency can lead to hypo….?

A

hypoCa, hypoK, hypoPTH

275
Q

Histology of pt w acute rheumatic fever

A

beady fibrous vegetations (verrucae), Aschoff bodies (granulomas) and Anitschkow cells (enlarged macrophages in granulomas)

276
Q

beady fibrous vegetation in acute rheumatic fever histology

A

verrucae

277
Q

enlarged cells within granulomas in acute rheumatic fever histology

A

anitschkov myocytes

278
Q

granulomas in acute rheumatic fever histology

A

Aschoff bodies

279
Q

what are signs of immunological phenomenon in infective endocarditis

A

glomerulonephritis, osler’s nodes, Roth spots, splenomegaly

280
Q

what are signs of emboli formation in infective endocarditis?

A

janeway lesions, splenic infarcts

281
Q

what is the gene mutation most assoc with adenocarcinoma of the lung?

A

EGFR mutation

282
Q

what are the immune therapies that bind to PD-1 to allow T cell activation?

A

Pembrolizumab and Nivolumab.

283
Q

what immune therapy binds to CTLA-4 to allow T cell activation? used to treat advanced metastatic melanoma

A

Ipilimumab

284
Q

what immune therapy binds to IgE and Is used in treating severe asthma?

A

omalizumab

285
Q

what antiviral is a pyrophosphate analogue that acts as a non competitive inhibitor of DNA polymerase and can be used to treat CMV infection?

A

foscarnet

286
Q

what cluster of differentiation is typically present on surface of a T cell to act as a costimulatory signal for activation of the T cell?

A

CD28

once CD80/86 binds to it.

287
Q

adenocarcinoma. pt treated w tyrosine kinase inhibitor which he did not respond to. what mutation is commonly assoc w this?

A

k-ras

288
Q

ejection fraction of a pt with long standing coronary heart disease falls after stent is inserted. why?

A

reperfusion injury.
due to oxidative stress, calcium overload, inflammation.
commonly, this leads to arrhythmias + biochemical abnormalities.
HF, indicated by a reduced ejection fraction, may last a few days but will normalise with sufficient treatment.

289
Q

hepcidin causes iron to accumulate in which cell?

A

macrophages

290
Q

what molecule is affected in TTP?

A

ADAMTS13 enzyme
a metalloprotease responsible for cleaving large multimers of vWF into smaller units.

-> inhibition of ADAMTS13 occurs in TTP

291
Q

defect in a5-chain of type IV collagen, resulting in eye and hearing abnormalities?

A

alports syndrome

x-linked

292
Q

IPEX syndrome- what gene involved?

A

foxp3.

  • this gene is required for the development of T regulatory cells. this condition is characterised by immunodeficiency, resulting in diarrhoea and eczema and endocrinopathies including T1DM and thyroid disease.
293
Q

what three sites are classically affected in hereditary angio-oedema?

A

upper airway, subcutaneous tissues and abdo organs (diarrhoea/ abdo pain).

294
Q

what antibodies are characteristically produced against a patient with primary membranous glomerulonephritis?

A

antibodies to phospholipase A2 receptor, a protein found on podocytes.

-> IC deposition on glomeruli and thus, nephrotic syndrome

295
Q

what does anti-cyclic citrullinated protein antibody bind to?

A

filaggrin - the filament aggregating protein

296
Q

type 2 autoimmune hepatitis- what autoantibody?

A

anti-LKM-1.

anti liver/ kidney microsomal-1 antibody

297
Q

anti centromere antibodies in the presence of Primary biliary cirrhosis indicates?

A

portal HTN

298
Q

high risk related to oculocutaneous albinism?

A
  • metabolic condition affecting the production of skin pigment melanin.
    higher risk of skin malignancy e.g. malignant melanoma.
299
Q

features of primary biliary cirrhosis

A

pruritus (increased bile salts in circulation).

reduced absorption of fat soluble vitamins e.g. A, D, E, K -> bruising/ night blindness/ osteomalacia.

300
Q

anti-CD11a antibody against CD11a on T cells, inhibits the migration of T cells. indicated in the tx of psoriasis

A

efalizumab

301
Q

rheumatoid arthritis

how does it affect the complement pathway?

A

high CH50.

complement factors are acute phase proteins and a high CH50 indicates acute or chronic inflammation.

302
Q

recurrent infections + addisons, hypothyroidism + hypoPTH

A

autoimmune polyendocrine syndrome Type 1. APECED syndrome: mutations in the AIRE gene.
dysfunctional parathyroid/ adrenal gland, hypothyroidism, gonadal failure, alopecia and vitiligo.

303
Q

common causes of non-igE mediated systemic histamine release

A

opioids, NSAIDs, contrast agents, exercise

304
Q

antibodies related to pemphigus vulgaris

A

anti-desmoglein 1 and anti-desmoglein 3

305
Q

initial encounter with antigen, mediated by which cell type?

A

antigen presenting cells such as macrophages engulf the allergen and presets peptides on the cell surface via MHC.

306
Q

initial encounter with antigen, what cytokine is released first?

A

CD4+ T cells recognize the peptide and bind to the macrophage acting as APC.
The macrophage then releases IL-12 which leads to production of memory CD4+ T cells.

307
Q

transplant from identical twin is called?

A

isograft

308
Q

brutons agammaglobulinaemia mx?

A

IVIG

309
Q

main cytokine responsible for fibrosis seen in CREST syndrome?

A

TGF-beta.

stimulates collagen production by fibroblasts.

310
Q

T1DM: autoantibodies to tyrosine phosphatase

A

anti-IA-2 and anti-phogrin abs.

311
Q

autoimmune polyendocrine syndrome. ptosis and difficulty w eye movement. diffuse pigmentation of the retina. hypoPTH and hypoCa.

A

Kearns-Sayre syndrome.

312
Q

epidermolysis bullosa caused by autoantibodies to?

A

type VII collagen.

bullae usually induced by trauma.

313
Q

axial rigidity/ stiffness assoc w hx of autoimmune disease and circulating anti-glutamic acid decarboxylase antibodies

A

stiff man syndrome.

314
Q

Guillain barre syndrome: what is the target for autoantibodies?

A

ganglioside LM1.

symmetrical inflammatory polyneuropathy that begins in the legs and ascends to involve motor neurone of the arms, face and muscles of respiration. pathogenesis involves cross reactivity between antibodies to the pathogen and components of peripheral n myelin components such as ganglioside LM1.

315
Q

pt presents w floaters and loss of accommodation in R eye, several weeks after experiencing trauma to L eye.

A

sympathetic ophthalmia, a granulomatous CD4+ T cell mediated disease.
trigger for the disease is trauma to damaged eye. the eye is an immunoprivileged site and is therefore, under normal circumstances, protected from possible autoimmune attack. Trauma to the eye breaks the tolerance -> increased photoreceptor antigen presentation to immune cells.

316
Q

how does skin act as an immune barrier.

A

tightly packed keratinized cells -> limits colonisation.
Low pH, low oxygen tension of skin. Sebaceous glands: hydrophobic oils repel water + microorganisms, lysozyme destroys structural integrity of bacterial cell wall, ammonia and defensins.

317
Q

how does secreted mucus act as an immune barrier?

A

Physical barrier.
Secretory IgA. Lysozyme and antimicrobial peptides. Lactoferrin act to starve invading bacteria of iron. Cilia- directly trap pathogens + contribute to removal of mucous, aided by physical manoeuvres such as coughing and sneezing.

318
Q

how does commensal bacteria act as an immune barrier?

A

Compete with pathogenic microorganisms for scarce resources. Produce fatty acids and bactericidins that inhibit the growth of many pathogens

319
Q

what do pattern recognition receptors recognize? e.g. Toll like receptors.

A

PAMPs - pathogen associated molecular patterns. e.g. bacterial sugars, DNA, RNA

320
Q

NADPH oxidase complex converts oxygen into what reactive oxygen species?

A

superoxide and hydrogen peroxide

321
Q

what does myeloperoxidase form from hydrogen peroxide and chloride?

A

HCl - highly oxidant and antimicrobial. part of oxidative killing.

322
Q

APC that resides in peripheral tissues

A

dendritic cells.

323
Q

characteristics of the adaptive immune system?

A

wide repertoire of antigen receptors, exquisite specificity, clonal expansion of cells w appropriate specificity, immunological memory.

324
Q

site of B cell maturation?

A

Bone marrow

325
Q

site of T cell maturation?

A

Thymus.

326
Q

secondary lymphoid organs?

A

Spleen, LNs, MALT

327
Q

what subset of T cells helps CD8 T cells and macrophages?

A

Th1 cells

328
Q

what subset of T cells express CD25+ and Foxp3+ and releases IL10 / TGFb

A

Treg cells

329
Q

B cell differentiation involves which ligands?

A

CD40-CD40L interaction

330
Q

what region of the immunoglobulin is associated with antigen recognition and binding?

A

Fab region

331
Q

What region of the immunoglobulin assoc w effector function?

A

Fc region

332
Q

how does the IL12-IFNy network usually work?

A

Infected macrophages produce IL12 -> IL12 induces T cells to produce IFNy -> IFNy stimulates macrophages to produce TNF and activates NADPH oxidase.

333
Q

Where is the mutation in X linked SCID?

A

Mutation of gamma chain of IL2 receptor on chromosome Xq13.1

334
Q

Very low or absent T and NK cell numbers, Normal or increased B cell numbers but low Igs

A

X linked SCID

335
Q

what is deficient in auto recessive SCID?

A 
Adenosine deaminase (ADA) deficiency
-> accumulation of precursors are toxic to lymphocytes.
336
Q

Auto recessive SCID immune cell numbers?

A

Very low or absent T, B and NK cell numbers

337
Q

what are the dysmorphic features of child with Digeorges syndrome?

A

High forehead, cleft palate, small mouth and jaw.

Low set, abnormally folded ears

338
Q

mx of auto recessive SCID?

A

PEG-ADA enzyme replacement therapy

339
Q

why does the oral route promote immune tolerance whereas the skin induces IgE sensitisation?

A

Ingestion of peanuts by mouth tends only to produce IgA or IgG response which are generally quite harmless. Food digested by the gut produces peptides of lower allergenity.
When skin barrier is disrupted (eg atopic dermatitis), allergen can access the dermis, where the dendritic cells can detect the allergen, causing release of cytokines and IgE antibodies

340
Q

what is the most common allergic disease in adults?

A

allergic rhinitis (20%)

341
Q

indications for specific IgE blood tests?

A

Patients who can’t stop anti-histamines

  • Patients with dermatographism
  • Patients with extensive eczema
  • History of anaphylaxis
  • Borderline/equivocal skin prick test results
342
Q

what is in an emergency community anaphylaxis kit?

A

epipen: preloaded adrenaline, prednisolone 20mg, and antihistamine tablet-cetrizine 10mg.

must call for ambulance and attend A+E after using emergency kit

343
Q

what is food associated exercised induced anaphylaxis?

A

Food induces anaphylaxis if individual exercises within 4-6 hours of ingestion. Common triggers = wheat, shellfish, celery.

344
Q

some indications for plasmapheresis?

A

when Ab itself is directly pathogenic. e.g. severe acute myasthenia gravis, severe vascular rejection of donor organ, goodpastures syndrome.

345
Q

what do calcineurin inhibitors do?

A

blocks IL-2 -> inhibit T cell activation and proliferation

346
Q

What is apremilast used for? mode of action?

A

effective in psoriasis, psoriatic arthritis.

PDE4 inhibitor.

347
Q

what is Tofacitinib used for?

mode of action?

A

Effective in rheumatoid arthritis.

Interferes with JAK-STAT signalling (JAK1 and JAK3 inhibitor)

348
Q

what does JC virus destroy?

A

infects and destroys oligodendrocytes

349
Q

Periodic fevers lasting 48-96 hours associated with: abdominal pain due to peritonitis, chest pain due to pleurisy and pericarditis, arthritis, skin

A

familial mediterranean fever.

mutation in MEFV gene - encoding pyrin-marenostrin.

350
Q

Mx of familial mediterranean fever?

A

Colchicine - binds to tubulin in neutrophils and disrupts function. Anakinra (IL-1R antagonist). Etanercept (TNFa inhibitor)

351
Q

long term risk of familial mediterranean fever?

A

amyloidosis.

due to extra deposition of AA protein (increased SAA- acute phase protein)

352
Q

APECED/ Autoimmune polyendocrine syndrome type 1 defect?

A

AIRE - autoimmune regulator

353
Q

What organs are affected in APECED- autoimmunity wise? and what infection most susceptible to?

A

parathyroid and adrenal glands - HypopTH and addisons.

+ candida infections.

354
Q

IPEX syndrome - mutation in?

A

Foxp3 - required for Treg cell development

355
Q

IPEX presentation?

A

endocrinopathy - T1DM, thyroid disease. diarrhoea, eczematous dermatitis

356
Q

ALPS (autoimmune lymphoproliferative syndrome) mutation in??

A

FAS pathway - failure of death of T lymphocytes

357
Q

ALPS (autoimmune lymphoproliferative syndrome) presentation

A

assoc w lymphoma.

high lymphocyte numbers w large spleen and LNs.

358
Q

what gene mutation is assoc w 30% of crohns disease?

A

NOD2 gene

359
Q

Tx of Crohns Disease

A

corticosteroid, azathioprine, anti-TNFa, anti-IL12/23

360
Q

Tx of Ankylosing Spondylitis

A

NSAIDs. Anti-TNFa, anti-IL17, antil-IL12/23.

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