Immuno Flashcards

1
Q

Reticular dysgenesis

Cause
Mutation
FBC findings

A

Failure of HSC differentiation
autosomal recessive adenylate kinase mutation
Everything is low except rbc

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2
Q

Kostmann syndrome

Severe congenital neutropenia

A

Failure of neutrophil precursors to differentiate
Autosomal recessive HAX mutation

Recurrent bacterial infections

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3
Q

Cyclic neutropenia

A

Failure of neutrophil differentiation
5 day period every three weeks where immunity fails and bacterial and fungal infections but good health in the interim
Autosomal dominant
ELA1 GENE

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4
Q

Leukocyte adhesion deficiency

A
CD18 deficiency ( beta 2 integrity)
Granulocytes can’t extravate into infected tissues
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5
Q

Investigations for innate immune system

A

FBC- NEUTROPHIL COUNT
Leukocyte adhesion marker assay
dihydrorhodamine flow cytometry- fluorescence means oxidative killing
NBT- nitroblue tetrazolium test- if it turns blue oxidative killing

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6
Q

Encapsulated bacteria you are susceptible to in complement deficiency

A
Strep pneumoniae 
Haemophilus influenzae 
Klebsiella pneumoniae
Neisseria meningitidus
Salmonella typhi 
Cryptococcus neoformams
Pseudomonas aerugosa
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7
Q

Common and terminal pathway deficiency

A

Cannot form MAC so susceptible to bacterial infections
CH50 and AP50 are abnormal
C3,5,6,7,8,9 are low

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8
Q

Classical pathway deficiency

A

C2 deficiency is common but C1q/r/s is also seen as is C4 deficiency
CH50 is abnormal
Associated with SLE because of CH50 abnormality

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9
Q

Alternative pathway deficiency

A

AP50 test is abnormal
Deficiency of factor P/I/B
Risk of infection with encapsulated bacteria

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10
Q

Lectin deficiency

A

MBL deficiency in 10%

Very common

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11
Q

SCID

A

X linked
Adenosine delaminates deficiency
Adaptive immunity disorder

Disorder in LYMPHOCYTE proliferation linked to IL2

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12
Q

Adaptive immune disorder

Di George syndrome

A
Cardiac dysfunction(ToF)
Abnormal fancies
Thymic aplastic
Cleft palate and oesophageal atresia
Hypocalcaemia due to low PTH

22Q11 DELETIONS

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13
Q

Adaptive immune disorder

Bare lymphocyte syndrome

A
Type 1 (rare) MHC I deficiency
Type 2 MHC II deficiency
In type 2 there are reduced CD4 cells and therefore reduced IgA and IgG as CD4 mediated class switching can’t occur
Recurrent viral infections
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14
Q

Type 1 hypersensitivity reaction

A

IgE mediated

Anaphylaxis, eczema

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15
Q

Type 2 hypersensitivity

A

Antibody against a cellular antigen
Churg -Strauss,goodpastures, graves

Do a Coombes test

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16
Q

Type 3 hypersensitivity

A

Antibody against immune complex

SLE, RA, polyarteritis nodosa

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17
Q

Type 4 hypersensitivity

A

T cell mediated
Delayed onset
Contact dermatitis, Graft rejection, T1DM

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18
Q

Foreign antigen rejection order

A

HLA DR >B>A

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19
Q

Acute graft rejection

A

Donor APC presents donor MHC/ antigen to host T cell

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20
Q

Chronic graft rejection

A

Host APC present donor MHC/ antigens to host T cell

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21
Q

Bare lymphocyte syndrome

A

Defect in regulatory factor x or class 2 transactivator

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22
Q

Type 1 bare lymphocyte syndrome

A

No CD8+ T cells

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23
Q

What condition is bare lymphocyte syndrome associated with and when does it present?

A

Associated with primary sclerosis cholangitis

Presents at 3 months of age

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24
Q

Why do lymphocytes fail to develop in BLS?

A

There is no HLA expression in the thymus so lymphocytes can’t develop

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25
Q

Why are there low mature T cells in di George syndrome?

A

The thymus is absent so can’t mature lymphocytes

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26
Q

What are the T cell mediated immune deficiencies

A

Di George syndrome

Bare lymphocyte syndrome

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27
Q

How to treat T cell mediated immune deficiencies

A

Thymus transplant in Di George syndrome
Infection prophylaxis
Complement replacement

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28
Q

Which CD factors to T cells require for activation?

A

CD80

CD86

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29
Q

What are the B cell mediated immune deficiencies

A

Selective IgA deficiency
Bruton’s agammaglobulinaemia
Combined variable immune deficiency
Hyper IgM syndrome

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30
Q

Brutons agammaglobulinaemia
B cell mediated disorder

Inheritance
Mutation
Presentation

A

No antibodies
BTK gene
X linked tyrosine kinase mutation
FAILURE TO PRODUCE MATURE B CELLS, SYMPTOMS AT 3-6 MONTHS

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31
Q

Common variable immune deficiency

B cell mediated

A

IgA,IgE, IgG are low ( think AGE)
B cells do not differentiate
Many causes

Presents as failure to thrive, granulomatous disease and autoimmunity

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32
Q

Elective IgA deficiency

A

1/600 Caucasians
Recurrent respiratory and gastro infections
Vaccination is helpful
70% asymptomatic

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33
Q

Ig pattern in hyper IgM syndrome

A

IgM is high
Low IgA and IgG

Because B cells not present to make the ig

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34
Q

Mutation and inheritance in hyper IgM syndrome

A

X linked
Xq26 mutation

CD154, CD40, CD40L AND AICDA defect

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35
Q

Mechanism of disease in hyper IgM syndrome

A

Activated T cells cannot activate B cells to class switch therefore IgA and IgG aren’t formed but IgM is.

Less lymphoid tissue as no germinal centre

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36
Q

What conditions are you susceptible to in B cell deficiencies

A

Tetanus
Diphtheria
Some viral and bacterial infections

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37
Q

Treatment of B cell deficiencies

A

BMT transplant
Ig prophylaxis
If IgA deficiency then vaccination

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38
Q

WBC in SCID

A

Low B cell
Low T cell
Low antibodies

No B cell can be normal

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39
Q

How do you diagnose kostmannsyndrome

A

1) chronically low neutrophil count

2) bone marrow scan showing arrest of neutrophil precursor maturation

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40
Q

Treatment of kostmann syndrome

A

G-CSF
BMT
prophylactic abx

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41
Q

Chronic granulotomous disease

A

Reduced oxidative killing because NADPH is defective

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42
Q

Chronic granulotomous disease test and treatment

A

NBT test

DHR test

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43
Q

Infections you are susceptible to if chronic granulotomous disease

A
Catalase positive organisms 
Pseudomonas
Listeria
Aspergillus 
Candida
E.coli
Staph aureus
Serattia
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44
Q

Treatment of chronic granulotomous disease

A

Prophylactic trimethoprim, itraconazole and interferon

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45
Q

Inflammatory cytokines

A

Il1
IL6
IL12
TNF

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46
Q

Anti inflammatory cytokines

A

TGF beta

IL10

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47
Q

What conditions are you predisposed to in cytokines deficiencies?

A

Salmonella
BCG
atypical mycobacterium
TB

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48
Q

What do cytokines do

A

They signal between T cells and macrophages
Stimulate NAPDH oxidative killing
Activate TNF

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49
Q

Treatment of anaphylaxis

A
Elevate legs
100%O2
IV was 100mcg hydrocortisone
Salbutamol
IM adrenaline 500mcg
IV chlorphenamine 10mcg
IV fluids
Seek help
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50
Q

Investigations in allergy

A

Ski. Prick test( positive test is wheal greater than 2mm than negative control- dilutant)
RAST ( check IgE response)
Component resolved diagnostics- tests specific IgE response to a specific allergen protein
Challenge test

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51
Q

What do you measure in an acute allergy reaction?

A

Mast cell tryptase (peaks 1-2 hours and baseline at 6 hours)

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52
Q

Limited cutaneous scleroderma

AKA CREST SYNDROME

A
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactily
Telangectasia

+ primary pulmonary hypertension

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53
Q

Antibody in crest syndrome

A

Anticentromere ab

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54
Q

Diffuse scleroderma

A

Crest+ pulmonary six+ GIT sx+ renal sx

Topoisomerase ab
Scl70 ab
RNA polymerase I, II, III ab
Fibrillarin ab

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55
Q

Sjorens syndrome ab

A

Anti ro and anti la

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56
Q

Sjorens syndrome symptoms

A

Dry mouth, dry eyes, PNS and pancreatic dysfunction

Keratoconjunctivitis sicca and xerostomia

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57
Q

IPEX syndrome

What does IPEX stand for

A

Immune dysregulation, polyendocrinopathy, enteropathy, X linked inheritance and autoimmune diseases

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58
Q

How is IPEX inherited

A

X linked recessive

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59
Q

Typical conditions in IPEX sufferers

A

Alopecia univeralis
Bulbous pemphagoid
Eczematous dermatitis
Nail dystrophy

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60
Q

Coeliac disease antibodies

What type of ab are they?

A

EMA
TGT

BOTH OF THESE ARE IgA

Antigliadin (IgG)

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61
Q

What condition is coeliac disease associated with (3)

A

Down’s syndrome
Dermatitis herpetiformis
T cell lymphoma

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62
Q

What HLA molecules are associated with with 95% of coeliac disease

A

DQ8

DQ2

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63
Q

What is the pathology in atopic dermatitis (infantile eczema)

A

Beta defensin defect which predisposes to staph aureus superinfection

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64
Q

Treatment for infantile eczema (atopic dermatitis)

A

PUVA phototherapy
Emollient cream
Steroid
Topical antibiotics

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65
Q

How to diagnose food allergy

A

Food diary
RAST
skin prick test
Food challenge

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66
Q

What is latex food syndrome

A

Foods such as avacado and chestnut have latex like properties so latex allergy sufferers get IgE mediated symptoms when they eat them

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67
Q

Signs of allergic rhinitis

A
Anosmia
Sneezing
Blueish nasal mucosa
Rhinitis
Nasal obstruction
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68
Q

Causes of acute urticaria

A

50% idiopathic
Viral illness
Febrile illness
Allergen

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69
Q

Pathology of acute urticaria

A

Resolves in 6 weeks
IgE mediated
Wheals

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70
Q

Component resolved diagnostics for peanut allergy

A

Ara h8 allergic to peanut and stone fruit but reaction is only oral
Ara h2- v allergic to peanuts

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71
Q

Pathology and diagnosis of haemolytic disease of the newborn

A

Path: maternal IgG mediated reticulocytosis and anaemia

Positive Coombs test

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72
Q

What is the antigen for HDN

A

Antigens on foetal rbc

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73
Q

Treatment of HDN

A

Maternal plasma exchange

Exchange transfusion

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74
Q

Evans syndrome

A

Autoimmune haemolytic anemia plus ITP

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75
Q

What is the pathology in AIHA

A

Complement mediated destruction of rbc
Also by Fcr, phagocytes and autoantibody

Anaemia

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76
Q

Diagnosis and treatment of AIHA

A

Positive Coombes test
Anti rbc autoantibody

Treat with steroids

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77
Q

Autoimmune thrombocytopenic purpura antigen (2) and antibody

A

Antigen glpIIb
Antigen: glp IIIa

Anti platelet antibody

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78
Q

Treatment of autoimmune thrombocytopenic purpura

A

IVIG
steroids
Splenectomy
Anti D antibody

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79
Q

Antigen for goodpastures syndrome

A

Non collagenous domain of basement membrane collagen type 4

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80
Q

Pathology of goodpastures syndrome

A

Pulmonary haemmorage and glomerulonephritis

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81
Q

Which immunoglobulin mediated goodpastures syndrome

A

IgG

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82
Q

Diagnosis of goodpastures syndrome (2)

A

Anti GBM antibody

Linear smooth IF Staining of IgG deposits on basement membrane

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83
Q

Antigen for pemphigus vulgaris

A

Epidermal cadherin

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84
Q

Which ig antibody mediates pemphigus vulgaris

A

IgG

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85
Q

Symptoms of pemphigus vulgaris

A

Non tense blistering of skin and Bullae

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86
Q

How do you diagnose pemphigus vulgaris

A

Immunoflourescance showing IgG deposition

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87
Q

Treatment of pemphigus vulgaris

A

Steroids

Immunosuppression

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88
Q

Graves’ disease

A

Anti TSH-R ab
Causes hyperthyroidismism
Treat with carbimazole and propylthiouricil

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89
Q

Myasthenia gravis antigen

A

Ach-R

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90
Q

Symptoms of myasthenia gravis

A

Muscle fatigue

Double vision

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91
Q

Diagnosis of myasthenia gravis

A

Tensilon test
EMG
anti ach-r antibodies

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92
Q

Treatment of myasthenia gravis

A

Neostigmine
Pyridostigmine
If severe IVIG and plasmapheresis

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93
Q

Antigen for acute rheumatic fever

A

M protein on group a strep

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94
Q

Symptoms, diagnosis and treatment of rheumatic fever

A

Myocarditis, Sydenham’s chorea and arthritis

Jones criteria to diagnose

Aspirin, penicillin and steroids

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95
Q

Antigen for pernicious anaemia

A

IF or gastric parietal cells

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96
Q

Symptoms and Diagnosis of pernicious anaemia

A

Low B12 and anaemia

Schilling test
Anti IF AB
Anti gastric parietal cell ab

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97
Q

Diagnosis of churg Strauss syndrome eGPA

A

P-ANCA against myeloperoxidase

Also see granulomas and eosinophilia granulocytes

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98
Q

Wegeners granulomatosis [GPA]

A

C-ANCA against proteinase 3

Granulomas

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99
Q

Symptoms of wegeners granulomatosis

A

Small vessel vasculitis
Cresenteric glomerulonephritis
Lung cavitations and pulmonary haemmorage
Sinus problems

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100
Q

Churg Strauss syndrome symptoms

A

Type 2 hypersensitivity

Triad of asthma, eosinophilia and vasculitis

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101
Q

Diagnosis of micropolyangiitis

A

P-ANCA against myeloperoxidase

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102
Q

Treatment of micropolyangiitis

A

Steroids
Cyclophosphamide
Azothioprine
Plasmapheresis

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103
Q

Symptoms of micropolyangiitis

A

Livido

Purpura

104
Q

Antigen for chronic urticaria

A
Cold 
Exercise
NSAID
Food
Pressure
Insect bites
Sun
Idiopathic
105
Q

Pathology for chronic urticaria

A

Anti IgE

IgG against FC§R1

106
Q

Diagnosis of chronic urticaria

A

Skin prick test
ESR (raised in urticarial vasculitis)
Challenge test

107
Q

Treatment of chronic urticaria

A
Avoid précipitants
Screen for thyroid disease
IM adrenaline for angiooedema ( associated with 50% of case)
1% menthol in aqueous cream for pruritis
Doxepin and cyclosporine May help
108
Q

Type II hypersensitivityndisorders (13)

A

Goodpastures Graves

Churgstrauss. Pemphigus vulgaris
Wegeners. Chronic urticaria
Micropolyangiitis
AIHA. Myasthenia gravis
HDN
EVANS SYNDROME. Rheumatic fever
AITP. Pernicious anaemia

109
Q

Type 3 hypersensitivity syndromes (4)

A

Mixed essential cryoglobulinaemia
Serum sickness
SLE
polyarteritis nodosa

110
Q

Human immunoglobulin

A

Give IV OR SC every 3-4 weeks
Hl is 18days
From 1000 donors
PREFORMED IGG AGAINST LOADS OF ORGANISMS

Give in pri wry and adaptive immunodeficiencies

111
Q

Specific immuniglobulin

A

Post exposure prophylaxis against rabies, tetanus, VZV and hep B

112
Q

Interferon alpha

Recombinant cytokine

A
Hairy celll leukoplakia 
Hep b and c
Kaposi sarcoma 
CML
Malignant myeloma
113
Q

Interferon beta as recombinant cytokine

A

Relapsing MS

114
Q

Interferon gamma (recombinant cytokine)

A

Chronic granulotomous disease

115
Q

Ipilimumab

A

Antibody for CTLA4
Indicated for advanced melanoma
Blocks the immune checkpoint and so enables Tcell activation

116
Q

Pembrolizumab

Nivolumab

A

ANTIBODY
Indicated for advanced melanoma
Enables T cell activation
Blocks immune checkpoint PD1

117
Q

Injection site reaction

A

Peaks at 48 hours

Usually CD8 cell infiltrate (NOT IGE)

118
Q

Antigens that cause transplant rejection

A

HLA DR>B>A

ABO

119
Q

Two stages of transplant rejection

A
  1. Recognition of foreign antigens
  2. Activation of antigen specific lymphocytes, proliferation of B cells and antibody production
  3. Effector phase (
    A.graft infiltration by all alloactive CD4 cells
    B.macrophages cause oxidative death and apoptosis
    C. cytottoxic T cells cause apoptosis by toxin release
    D. antibodies bind to graft endothelium,
120
Q

How do you match transplant cases

A
  1. ABO
  2. HLA MATCH VIA PCR
  3. check preformed antibodies with cdc, facs and luminex
  4. Cross match and check if recipient serum will react with donor lymphocytes using facs and luminex
  5. check for antibodies to the graft after transplantation
121
Q

What are cdc, facs and luminex

A

Cdc: complement dependent cytotoxicity
Facs: flow cytometry
Luminex: flow cytometry but for solids and picks up individual HLA reactivities

122
Q

What are the pre transplant induction agents that suppress T cell responses

A

Anti CD25 basiliximab
ANTI CD52 alemtuzumab
OKT3
ATG

123
Q

How to prevent transplant rejection

A
  1. CNI
  2. MMF/AZO
  3. STEROIDS
124
Q

How to treat acute transplant rejection

A

CELLULAR
steroids, OKT3, ATG

Antibody
IVIG, plasma exchange, anti CD5, anti CD20

125
Q

GVHD prophylaxis

A

Cyclophosphamide

Methotrexate

126
Q

GVHD symptoms and treatment

A

Skin rash
Gut pain, N&V, bloody diarrhoea, jaundice

Treat with steroids

127
Q

Haematopoeitic stem cell transplant steps

A
  1. Total body irradiation plus cyclophosphamide
  2. Repack BM with donor BM
  3. Reaction of DONOR LYMPHOCYTES against HOST TISSUE
128
Q

Infection risk after transplant

A

CMV
BK VIRUS
PNEUMOCYSTITIS CARINII

129
Q

Malignancy risk post transplant

A

Kaposi sarcoma due to HSV8
SKIN CANCER
LUNG CANCER

130
Q

Atherosclerosis risk post transplant

A

Hypertension and hyperlipidaemia cause MI risk to increase 20 times compared to matched controls

131
Q

HIV antigens

A

Glp120 for initial binding to CD4 cells
Glp41 which induces conformational change in CD4 cells
CCR5 and CXCR4 chemokine coreceptors to infect macrophages
Gag protein for HIV virus structural support

132
Q

Innate immune response to HIV virus

A

Macrophages, NK cells and complement are activated
Dendritic cell stimulated via TLR
Cytokine and chemokine release

133
Q

Adaptive immune response to HIV

A

Neutralising antibodies: anti gp120, anti
Non neutralising antibodies: anti p24, gag antibodies

Chemokine produce MIP1a and MIP1b plus RANTES which block coreceptors

134
Q

How does HIV damage the immune hresponse

A
  1. Disables CD4 cells
135
Q

Lifecycle of HIV

A
  1. Attachment and entry
  2. Reverse transcription
  3. Viral transcription
  4. Viral integration
  5. Synthesis of viral proteins
  6. Assembly of virus
  7. Maturation
136
Q

What predicts HIV viral progression

A

Initial viral set point

137
Q

What is the normal time course from HIV infection to AIDS

A

8-10 years

138
Q

What percentage are long term non progresseds with a stable viral loadin HIV

A

Less than 5%

139
Q

What are rapid progressors in HIV

A

The 10% of people who progress to AIDS in 2-3 years

140
Q

What is the screening test for HIV

A

ELISA for anti-HIV antibodies

141
Q

What is the confirmation test for HIV

A

Western blot for HIV ab

142
Q

How long does it take for HIV seroconversion to happen

A

About 10 weeks

143
Q

How is viral count calculated in HIV

A

PCR

144
Q

How is CD4 count measured in HIV

A

FACS

145
Q

Treatment of HIV

A

HAART

efavirenz, tenofivir, emtricitabine

146
Q

Treatment of HIV in pregnancy

A

Ziduvidine

147
Q

Limitations of HAART

A

Difficult adherence and high pull burden
Doesn’t restore specific HIV T cell response
Doesn’t eradicate latent HIV1
Toxicities

148
Q

HAART

fusion inhibitors

A

Enfuviratide

149
Q

Enfuvirtide SE

A

Local reaction

150
Q

HAART

attachment inhibitors

A

Maraviroc

151
Q

Nucleoside reverse transcriptase inhibitors

A
Abacivir
Combivir
Didanosine
Emtricitabine
Emzicom
Lamivudine
Stavudine
Tramivir
Zalcitabine
Zidovudine
152
Q

HAART therapies causing peripheral neuropathy

A

Zalcitabine and stavudine

153
Q

reverse transcriptase inhibitors causing GI upset and fever

A

Zidovudine

154
Q

Reverse transcriptase inhibitors causing allergic reaction

A

Abacavir

155
Q

Nucleoside reverse transcriptase inhibitors causing mitochondrial toxicity

A

Stavudine

156
Q

NucleoTide reverse transcriptase inhibitor

A

Tenofivir

157
Q

NucleoTide reverse transcriptase inhibitors causing BM toxicity

A

Tenofivir

158
Q

HAART therapy

Integration inhibitors

A

Raltegravir

Elvetegravir

159
Q

HAART therapy

Protease inhibitors

A

Amprenavir
Fosamprenavir

Lopinavir
Saquinavir
Nelfinavir
Ritonavor
Indinavir
160
Q

SE of protease inhibitors

A

Hyperlipidaemia
Fat redistribution
T2DM

161
Q

Efavirenz nonNRTI SE

A

CNS effects

162
Q

Nevirapine (non NRTI) SE

A

Hepatitis and rash

163
Q

Delavirdine (nonNRTI) SE

A

rash

164
Q

Where are central memory T cells found

A

Tonsils and lymph nodes

165
Q

How do central memory cells travel

A

They roll along and extravasate in high endothelial venules (HEV)

166
Q

What antigens are present on central memory cells and how do they help

A

CCR7
CD62L

These allow entry to HEV and then to peripheral lymph nodes

167
Q

What cytokine do central memory cells produce

A

IL2

168
Q

Central memory cells tend to be found in…

A

The CD4 population

169
Q

Where are effector memory cells found

A

Lung liver and gut

170
Q

What do effector memory cells produce

A

Perforin and IFN gamma

171
Q

Effector memory cells tend to be found in the ….

A

CD8 population

172
Q

What does CCR7 do

A

It binds to CCL19 & CCL21 on the luminal surface of endothelial cells of lymph nodes and causes firm arrest and starts the process of extravasion

It causes the homing of dendritic cells to lymph nodes

173
Q

What does cd62l do

A

It interacts with a molecule on HEV to mediate attachment and rolling

174
Q

Which cytokines are involved in Th2 response

A

IL 4,5,6

175
Q

Which cytokines are involved in Th1 response

A

IL2, IFN GAMMA AND TNF

176
Q

What are adjuvants in vaccines for

A

Slowing the release of antigen

177
Q

What is ALUM (vaccine adjuvant)

A

Antigens are adsorbed to alum to ensure they are released slowly.

It activates gr1+ cells to produce IL4 to prime naive B cells

178
Q

Adjuvants for vaccines

A
Alum
Cpg
Complete freund’s adjuvant
ISCOMS
IL2
179
Q

What is ISCOMS

A

Expeiemental multimeric antigen+ adjuvant

When combined with saponin their is a strong serum response

180
Q

How is IL2 used as an immune adjuvant

A

In individuals with Hep B surface antigen it is used tomforce seroconversion

181
Q

What is Cpg (vaccine adjuvant)

A

It activates TLRs on APC to increase costimulatory activity

Immunostimulatory activity is linked to unmethylated DNA motif (where cytosine is next to guanine)

182
Q

What is complete freund’s emulsion and is it used in humans

A

It is an oil in water solution containing mycobacterium cell wall
It is used only in animals

183
Q

Which are the passive vaccines

A
Hep A
Hep B
Measles
Rabies
RSV
184
Q

What is the Mantoux test

A

Intradermal injection of 0.1ml of 5 units of tuberculin.
If after 72 hours a swelling of greater than 10mm is felt then there has been previous TB exposure, either via mantoux or by infection

185
Q

Antigen in mixed essential cryoglobulinaemia

A

IgM against IgG +/- hep C

186
Q

Antigen in serum sickness

A

Reactions to proteins such as Penicillin in anti serum

187
Q

Antigen in polyarteritis nodosa

A

Hep B, hep C

188
Q

Pathology in SLE

A

Anti dis-DNA and anti sm

189
Q

Antigen for T1DM

A

Pancreatic beta cell proteins such as GAD (glutamate decarboxylase)

190
Q

Antigen and pathology of MS

A

Myelin proteins such as MBP and proteolipid protein

IgG oligoclonal bands on electrophoresis

191
Q

Treatment of MS

A

Interferon beta

Steroids

192
Q

Antigen and antibody in rheumatoid arthritis

A

Antigen is protein in synovial membrane
Anti CCP antibody
Rheumatoid factor used for diagnosis

193
Q

Gene mutation in 30% of Crohn’s disease

A

NOD2

Chromes is Th1 mediated

194
Q

HLA association for ankylosis spondylitis

A

HLA B27

195
Q

HLA association for goodpastures syndrome

A

HLA DR2/15

196
Q

HLA association for Graves’ disease

A

HLA DR3

197
Q

HLA association for SLE

A

HLA DR3

198
Q

HLA association for T1DM

A

HLA DR3/4

199
Q

HLA association for rheumatoid arthritis

A

HLA DR4

200
Q

What is genetic polymorphism PTPN22

A

A mutation in lymphocyte tyrosine phosphatase associated with RA, T1DM and SLE

201
Q

What is polymorphism CTLA4

A

T cell receptor for CD80 and CD86 which transmits inhibitory signals for T cell activation. Associated with autoimmune thyroid disease, SLE and T1DM

202
Q

Autoantibody in antiphospholipid syndrome (Hugh’s syndrome)

A

Antibodies against cardiolipin and beta 2 glycoproteins and lupus anticoagulant

203
Q

Autoantibody in autoimmune hepatitis

A

Anti smooth muscle antibody
Anti liver kidney microsomal 1 antibody
Anti soluble liver antigen

204
Q

Autoantibody of infants with complete heart lock from mothers with SLE

A

Anti ro

205
Q

Dermatomyositis autoantibody

A

Anti Jo1

206
Q

Autoantibody in Hashimoto’s thyroiditis

A

Anti thyroglobulin and anti thyroperoxidase

207
Q

Autoantibody in mixed connective tissue disease

A

Anti U1RNP

208
Q

Autoantibody in dermatomyositis

A

Anti Jo1

t RNA synthetase

209
Q

Auto antibody for primary biliary cirrhosis

A

Anti mitochondrial antibody

210
Q

Prednisolone mechanism of action

A

Phospholipids A2 inhibition to block arachadonic acid and therefore prostaglandin production
Inhibits phagocyte trafficking, release of proteolytic enzymes and phagocytosis
Blocks cytokine gene expression
Decrease ab expression
Promotes cell apoptosis

211
Q

SE of prednisolone

A
Cushingoid symptoms (adrenal suppression)
Avascular necrosis
Osteoporosis
Transient neutropenia
Peptic ulceration
212
Q

Any proliferative agents

Cyclophosphamide:moa

A

Alkylate guanine of DNA, so damage DNA replication. Works better on B than T cells but at high doses affects all cells

213
Q

SE of cyclophosphamide

A
Teratogenic
BM suppression
Hair loss
Haemmoragic cystitis
Infertility
214
Q

Indications for cyclophosphamide

A

Anti cancer
Vasculitis
Connective tissue agent

215
Q

Plasmapheresis mechanism

A

Aim: removal of a pathogenic stimulus

Blood removed and immunoglobulins removed and then reinfused. If replaced with albumin then it is plasma exchange

216
Q

Plasmapheresis indications

A

Antibody mediated disease and reactions e.g. antibody mediated transplant rejection

SE include anaphylaxis and rebound low antibody

217
Q

Methotrexate mechanism

A

Inhibits dihydrofolate reductase and so black,s DNA replication

218
Q

Methotrexate Se

A
Infection, BM suppression, 
Teratogenic 
Pulmonary fibrosis
Hepatotoxicity
Folate deficiency- megaloblastic macrocytic anaemia
219
Q

Azothioprine indications

A

Transplant
Autoinflammatory conditions
Autoimmune disease

220
Q

Azothioprine mechanism

A

Metabolised by liver to 6 mercaptopurine and inhibits de novo purine synthesis.
Blocks T cell activation

221
Q

Methotrexate indications

A

Crohn’s disease
RA
psoriasis
Abortifacients

222
Q

Anti proliferative agent

Mycophenalate mofetil

A

Blocks de novo Nucleotide synthesis by preventing guanine sysnthesis
Does this by blocking IM PDH

223
Q

Immune therapies

Inhibitors of cell signalling

A
Tacrolismus
Cyclosporin
Sirolimus
Tofacitinib
Apremilast
224
Q

Apremilast

A

PDE4 INHIBITOR

For psoriasis

225
Q

Tocafatinib

A

JAK inhibitor For RA

226
Q

Sirolimus

A

Blocks clinal proliferation of T cells and is used as anti rejection meds

227
Q

Inhibits Calineurin to prevent IL2 transcription and therefore block the T cell response

A

Tacrolismus
Cyclosporin

Anti rejection meds for transplant

228
Q

SE of tacrolismus and cyclosporin

A

Nephrotoxic, hypertension,neurotoxicity

Cyclophosphamide also causes gingival hypertrophy

229
Q

Basaliximab

Agent directed at cell surface antigens
block signalling, cell depletion

A

Anti CD25- alpha chain of IL2 receptor and inhibits T cell activation

Used in allograft rejection

SE
GI disturbance, infection, malignancy and local site reaction

230
Q

Abatacept

Agent directed at cell surface antigens
block signalling, cell depletion

A

Anti CTLA4-ig

used for RA

SE cough, infection, malignancy, infusion reaction

231
Q

Rituximab

Agent directed at cell surface antigens
block signalling, cell depletion

A

Anti CD20 so depletes B cells

SLE, RA, lymphoma

SE infection, exacerbates CVS disease

232
Q

Natalixumab

Agent directed at cell surface antigens
block signalling, cell depletion

A

Anti alpha 4 integrin that binds to VCAM1 and MADCAM1 to mediate rolling and array of leukocytes

Used in RRMS, Crohn’s disease

SE hepatotoxic, infection, PML, malignancy and infusion reaction

233
Q

Tocilizumab

Agent directed at cell surface antigens
block signalling, cell depletion

A

Anti IL6 so prevent macrophage, B, T cell and neutrophil activation

Used in castle and disease and RA

SE
Infusion reaction, hyperlipidaemia, hepatotoxic

234
Q

Muromonab CD3

Agent directed at cell surface antigens
block signalling, cell depletion

A

Mouse ab (OKT)

Blocks CD3 on T cells

Active anti allograft rejection med

SE fever, leukopenia

235
Q

Anti thyomite globulin (ATG)

Agent directed at cell surface antigens
block signalling, cell depletion

A

Allograft rejection med esp renal or heart transplant

It modulates T cell activation and proliferation. It causes leukocyte depletion

SE infusion reaction, infection, malignancy, leukopenia

236
Q

Daclizumab

Agent directed at cell surface antigens
block signalling, cell depletion

A

Anti IL2 receptor antibody that targets CD25

Organ transplant rejection prophylaxis

237
Q

Efalizumab

Agent directed at cell surface antigens
block signalling, cell depletion

A

Anti CDIIa which inhibits migration of T cells

238
Q

Alemtazumab (campath)

Agent directed at cell surface antigens
block signalling, cell depletion

A

Mab that binds to CD52 on lymphocytes to cause depletion

Used in RRMS, CLL

SE risk of CMV infection

239
Q

Anti TNF alpha agents

A

Infliximab
Adalimumab
Certolizumab
Golimumab

240
Q

Indications and SE of anti TNFalpha therapy

A

RA, IBD, ANK SPOND, PSORIASIS

SE
Infections eg hepatitis B/C/TB
Demyelination 
Lupus like conditions
Lymphoma
241
Q

Etanercept

Blocks action of cytokines

A

TNF alpha/ beta p75 IgG fusion protein

Used in RA, ank spond and psoriasis

SE

Infections eg hepatitis B/C/TB
Demyelination
Lupus like conditions
Lymphoma

242
Q

Ustekinumab

Anti cytokine

A

Blocks IL12 AND IL23( binds to p40 subunit)

Used for psoriasis

SE
cough, injection site reaction, malignancy, infection (TB)

243
Q

Secukinumab

Anti cytokine

A

Anti IL17a

Used for psoriasis

SE TB infection

244
Q

Denusumab

A

Anti RANK ligand antibody. It prevents RANK mediated osteoclasts differentiation and recruitment.

Used in osteoporosis, multiple myeloma, bony metastasis

SE
avascular necrosis of jaw and infection

245
Q

Hyperacute transplant rejection

A

Complement mediated activated by preformed antibodies

Results in thrombosis and necrosis and occurs within hours

Treat by HLA cross matching

246
Q

Acute transplant rejection (cellular)

A

A type 4 hypersensitivity reaction (cell mediated) occurring over weeks. Caused by CD4 cells

Treat with T cell suppression

247
Q

Acute transplant reaction ( antibody mediated)

A

Occurs over months
Due to B cell activation where antibody attacks vessels causing vasculitis

Treat with Ab removal and B cell suppression

248
Q

Chronic graft rejection

A

RF: HTN, previous rejectionS, hyperlipidaemia

Sx: bronchiolitis obliterans, glomerulopathy, vasculopthy, fibrosis

Tx: try and reduce end organ damage

249
Q

GVHD

A

Donor cells attacking the host- occurs over days to weeks
Symptoms include rash, jaundice, D&V

Treat with steroids and immunosupression

250
Q

features of chronic granulomatous disease

A

absent respiratory burst because of reduced NADPH and impaired oxidative killing

excessive inflammation and persisting neutrophils because antigens aren’t cleared
granuloma formation
lymphadenopathy and hepatosplenomegaly

251
Q

what cytokines are deficient in cytokine deficiency

A

IL12/ IL12R

IFNg/ IFNgR

252
Q

what infections are you predisposed to in cytokine deficiency

A

salmonella

mycobacteria

253
Q

infections you are predisposed to in phagocyte deficiency

A

bacteria: staph aureus, enteric
mycoplasma: TB, atypical mycobacteria
fungi: candida, aspergillus flavus/ fumigatus

254
Q

what is complement

A

proteins produced in liver that circulate as inactive molecules
when activated cause a cascade via enzyme action

255
Q

SLE is associated with a defect in which complement pathway

A

classical

256
Q

how does the classical complement pathway clear immune complexes

A

via erythrocytes

deficiencies cause skin/ joint inflammtion