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Year 5 Pathology > Immuno > Flashcards

Flashcards in Immuno Deck (176):
1

Allergic rhinitis

Loss smell, nasal itching and discharge, swollen and hyperemic nasal mucosa.
Tx with corticosteroid spray (fluticasone) +/- PO antihistamines

2

Hereditary angioedema

Laryngeal edema, abdo sxs (pain, D&V, constip, distension), ?swelling hands and feet.
Triggered by infections, stress, menstruation, ACEI

3

Acute urticaria

4

Urticarial vasculitis

Ecchymoses and hyperpigmentation may occur in healing process
Biopsy for dx

5

Classic urticaria reaction

Latex

6

Which Ig is a dimer?

IgA

7

Which Ig is most abundant?

IgG

8

Chemotactic agents

IL8, LTB4, C5a, bacterial products, kallikrein

9

Kostmann's

Congenital neutropenia

10

Examples of Type II HSR

Hemolytic anemias
Graves
Goodpastures
ITP
Myasthenia Gravis
Type I DM

11

Examples Type III HSR

SLE
HCV-associated MPGN
RA
Polyarteritis nodosa
Arthus reaction
Serum sickness

12

c-ANCA

Wegener's

13

p-ANCA

Polyarteritis nodosa
Microscopic polyangitis

14

Live vaccines

MMR
BCG
Yellow fever
Oral polio (Sabin)
VZV

15

Killed vaccines

Rabies
Influenza (IM)
Polio (salk)
HAV

16

Subunit/congugate vaccine

Pneumococcal

17

Congugate vaccine

Hib

18

Recombinant vaccine

HBV

19

Detoxified vaccine

Tetanus

20

Inactivated bacterial preparation vaccine

Whole cell typhoid

21

Alum

Agent that produces predominantly Ab response through release IL-4 which primes naive B cells

22

Vaccination: active or passive immunity

Active

23

Immune globulin: active or passive immunity

Passive

24

Innate immunity composed of?

Epithelium
Mucus
Complement
Myeloid cells: granulocytes, macros, mast cells

25

TLRs

On cells of innate immune system.
Recognize PAMPs, e.g. CD14 (on macros) is a TLR that recognizes LPS on gram neg bacteria

26

TLR activation

Upregulates NF-KB

27

NF-KB

"On switch for acute inflamm response"

28

Which products of AA mediate vasodilation and increased vascular permeability?

PGI2, PGD2, PGE2

29

Mediate neutrophil chemotaxis

LTB4, IL8, C5a, bacterial products, kallikrein

30

Which AA products mediate vasoconstriction, bronchoconstriction, and increased vascular permeability?

LTC4, LTD4, LTE4

31

Activators or mast cells

Tissue trauma
C3a, C5a
Cross linking of IgE by antigen

32

Immediate response of mast cells

Histamine release:
-arteriole vasodilation
-increased vascular permeability at post-cap venule

33

Delayed response of mast cells

Production AA metabolites, esp LTs

34

Classical pathway complement

C1 bings IgG or IgM that is bound to an antigen

35

Alternative pathway complement

Microbial products directly activate

36

Mannose lectin binding pathway complement

MBL binds mannose on microorganisms

37

All complement pathways meet where

Production C3 convertase, C5 convertase

38

Complement proteins that trigger mast cell degranulation

C3a, C5a

39

Complement protein that acts as opsonin for phagocytosis

C3b

40

Hageman factor

Inactive pro-inflamm protein produced by liver.
Activated on exposure to subendothelial or tissue collagen
Activates coag and fibrinolytic systems, complement, kinin system

IMPORTANT IN DIC!!!

41

Bradykinin functions

Vasodilation
Increased vascular permeability
Pain

42

Pain mediators

Bradykinin, PGE2
Sensitize sensory nerve endings

43

Rubor and Calor

Due to vasodilation causing increased blood flow
HISTAMINE, PGs, bradykinin

44

Tumor

Leakage fluid at post-cap venule into interstitial space
Histamine, tissue damage

45

Fever mechanism

Pyrogens cause release IL1, TNF which increase COX activity in perivascular cells of hypothalamus. Increased COX=increased PGE2 which increases temp set point

46

Steps of neutrophil arrival

1. Margination
2. Rolling
3. Adhesion
4. Transmigration
5. Phagocytosis
6. Destruction phagocytosed material

47

Rolling-mechanism

Selectin speed bumps.
P selectin from Weibel Palade bodies, histamine mediated.
E selectin induced by TNF, IL1

48

Adhesion-mechanism

Cellular adhesion molecules (CAMS) (on endothelium) and integrins (on neutrophils) bind

49

Upregulators CAMS

TNF
IL-1

50

Upregulators integrins

C5a, LTB4

51

Leukocyte adhesion deficiency

AR defect in CD18 subunit of integrin
Thus no adhesion
Sxs/signs: delayed separation umbilical cord, neutrophilia, recurrent bacterial infections without pus

52

Chediak Higashi disease

Protein trafficking defect (microtubules)-phagosome can't bind with lysosome
Sxs:
Pyogenic infections
Neutropenia (can't divide)
Giant granules by Golgi
Defective primary hemostasis
Albinism
Peripheral neuropathy

53

O2 dependent killing

Most effective
Generates HOCl (O2 -> superoxide ->H2O2 ->HOCl

54

O2 independent killing

Less effective
Via enzymes in leukocyte: lysozyme, major basic protein

55

CGD

NADPH oxidase defect
Infections and granulomas with catalase positive organisms
Nitro-blue tetrazolium test (NBT) remains colorless

56

Catalase positive organisms

S aureus
Pseudomonas cepacia
S marcescens
Nocardia
Aspergillus

57

MPO deficiency

MPO converts H2O2 to HOCl.
In theory similar to CGD but reality: only increased risk Candida

58

When in inflamm do macros peak

2-3 days after start inflamm

59

When in inflamm do neutrophils peak

24 hrs after start inflamm

60

How do macros get from blood into periphery

Same way neutrophils do (margination, rolling, adhesion, transmigration)

61

Method by which macrophages kill

O2 INdepedent, esp lysozyme

62

Outcomes acute inflamm

Resolution and healing (IL10, TGF-B)
Continued acute inflamm (macros call in more neuts via IL-8)
Abscess formation
Chronic inflamm

63

Chronic inflamm cells

Lymphos and plasma cels

64

Stimuli for chronic inflamm

Persistent infection (MCC)
Viruses, mycobacteria, parasites, fungi
AI
Foreign material
Some cancers

65

T cell activation requirements

1. Binding antigen/MHC complex
2. Secondary signal

66

Types CD4+ cells

TH1, TH2

67

TH1 products

IL2, IFN-gamma

68

IL2

T cell growth factor

69

IFN-gamma

Macrophage activator

70

TH2 products

IL4
IL5
IL10

71

IL4

IgE

72

IL5

IgA

73

IL10

Inhibits TH1

74

CD8+ cells

Perforins, granzyme
Express FasL which binds Fas on target cell

75

First Igs on B cell

IgD, IgM

76

Granuloma-key feature

Epitheliod histiocyte

77

Ddx-non-caseating granuloma

Foreign material, e.g. leaking breast implant
Sarcoidosis
Berylliosis
Crohns
Cat scratch (stellate shaped)

78

Ddx-caseating granuloma

TB
Fungus

79

DiGeorge syndrome

Developmental failure 3rd and 4th pharyngeal pouch
22q11 microdeletion
T cell deficiency, hypocalcemia, abnormalities great vessels, heart, and face

80

T cell deficiency symptoms

Viral and fungal infections

81

B cell deficiency symptoms

Bacterial, protozoal infections

82

3rd pharyngeal pouch

Inferior parathyroids, thymus

83

4th pharyngeal pouch

Superior parathyroids

84

SCID

Defective cell-mediated and humoral immunity. All the infections, including opportunistic.
Etiologies:
1. Cytokine receptor defects
2. Adenosine deaminase deficiency
3. MHC class II deficiency

Tx-sterile isolation, stem cell transplant

85

Cell-mediated immunity

T cells

86

Humoral immunity

B cells

87

X linked agammaglobulinemia

Complete lack Igs due to disordered B cell maturation
Bruton tyrosine kinase (BTK) mutation
Infections with bacteria, enterovirus, Giardia
Avoid live vaccines

88

Common variable deficiency

Low Ig due to B cell or TH cell defects
(Exams: mutation MHC III)
Bacteria, enterovirus, Giardia infections
Increased risk AI disorders, lymphoma

89

Which two immunodeficiencies result in bacterial, enterovirus, and Giardia infections?

Common variable
X linked (Bruton) agammaglobulinemia

90

Most common Ig deficiency

IgA

91

IgA deficiency

Mucosal infections, esp viral.
NB: celiacs tend to have this a lot

92

Hyper-IgM syndrome

Mutation in CD40L or CD40R...problem with class switching-no 2nd signal.
Decreased IgA, IgG, IgE causes recurrent infections, esp mucosal

93

Wiskott Aldrich syndrome

Defect in WASP (Wiskott-Aldrich Syndrome Protein)
Thromboytopenia, eczema, recurrent infections (humoral and cell mediated)
X linked

94

C5-C9 deficiency

Neisseria infections

95

C1 inhibitor deficiency

Hereditary angioedema (esp periorbital edema).
Excess activation complement (therefore vasodilation and increased vascular permeability)

96

Primary mech for autoimmunity

Loss of self tolerance: many self-reactive lymphos regularly generated but undergo TOLERANCE (central and peripheral). E.g. negative selection in thymus causes self-reactive lymphos to undergo apoptosis

97

AIRE mutation

AI polyendocrine syndrome (APECED): hypoparathyroidism, adrenal failure gonadal failure, alopecia, vitiligo, candida.
Failure of negative selection: no AIRE to express "self".
AIRE is also called "autoimmune regulator"

98

Central tolerance in bone marrow

Negative selection: if bind "self" too strongly, either undergo receptor editing or apoptosis

99

Peripheral tolerance

In blood. Anergy (no second signal) or apoptosis via Fas death receptor

100

ALPS

Autoimmune Lymphoproliferative Syndrome
Mutation in Fas apoptotic pathway
Cytopenias, LAD, HSM (LAD and HSM due to proliferation lymphos....can lead to lymphoma)

101

FOXP3 mutation

IPEX syndrome

102

IPEX syndrome

Immune dysfn Polyendocrinopathy Enteropathy X linked
Polyendo--thyroiditis, diabetes
Enteropathy--diarrhea

103

CD25 polymorphisms

Associated with autoimmunity, e.g. MS, Type I diabetes

104

HLA B27

Ankylosing spondylitis

105

Environmental triggers for autoimmunity

Infection (self reactive activated)
Molecular mimicry (antigen resembles self-antigen)

106

Reason for female bias in autoimmunity

Esrogen decreases tolerance, therefore more self-reactive lymphocytes

107

SLE

Type III HSR
Decreased levels CH50, C3, C4
ANA, anti-dsDNA, anti-Sm

108

Drug induced lupus

Hydralazine, procainamide, isoniazid
Anti-histone Abs
CNS, renal involvement rare

109

Antiphospholipid syndrome

Abs + hypercoagulable state
Lifeling anticoagulation--arterial and venous thromboses
Can occur with SLE but more commonly is primary disorder

110

Sjogrens

AI destruction lacrimal, slaivary glands
Type IV HSR
Anti-SSA, SSB, ANA, RF
Increased risk B cell lymphoma (U/L enlargement parotid)

111

Systemic sclerosis

Fibroblast activation causing collagen deposition. Massive amounts fibrosis
Limited type (CREST) and diffuse type

112

CREST syndrome antibody

anti-centromere

113

Diffuse systemic sclerosis Ab

anti-topoisomerase I

114

Mixed connective tissue disease--Antibodies

Anti U1 RNP antibodies

115

Labile tissues

Continuously cycle to regenerate tissue.
Small and large bowel
Skin
Bone marrow

116

Stable tissues

Quiescent but can re-enter cell cycle.
LIVER

117

Permanent tissues

Heal by repair; no regenerative capacity
Myocardium
Skeletal muscle
Neurons

118

Initial phase repair

Formation of granulation tissue

119

Granulation tissue mediators

Fibroblasts, capillaries, myofibroblasts

120

Scar formation

Result of repair
Type III collagen replaced by Type I

121

Causes delayed wound healing

Infection (MCC)
Vit C, Cu, Zn deficiency
Foreign body/continued inflamm, diabetes, ischemia, malnutrition

122

Hypertrophic scars

Excess production of Type I collagen

123

Keloid scars

Excess production scar tissue out of proportion to wound
Type III collagen
*Earlobe

124

Bare lymphocyte syndrome

Deficiency in MHC I or MCH II
Low IgA, IgG
Associated with sclerosing cholangitis (hepatomegaly, jaundice)

125

IFN-gamma deficiency

Can't form granulomas
TB
And salmonella, for some reason

126

OKT3

Aka muromonab.
Monoclonal antibody (mAb) against CD3.
Treats rejection episodes in transplant pts, by clearing T cells from circulation.
Use when acute steroid-resistant rejection of heart, liver, kidney transplants

127

Cyclosporin
-MOA
-SE

Inhibits calcineurin (therefore T cells)
SE: gum hypertrophy

128

Azathioprine
-Mech

Metabolized to 6MP. Prevents DNA synthesis, esp lymphocytes

129

Sirolimus

Aka rapamycin
Inhibits T cell prolif by binding FK-binding protein 1A (FKBP-1a)

130

Anti-Jo1

Dermatomyositis

131

Anti-smooth muscle

AI hepatitis

132

Anti-centromere

CREST syndrome

133

Anti-topoisomerase I

Diffuse scleroderma

134

Antimitochondrial

PBC

135

Anti-glutamic acid decarboxylase

T1DM

136

Anti-endomysial

Celiac

137

Anti-cardiolipin

Antiphospholipid
SLE
Syphilis

138

Goodpastures

Against Type IV collagen.
Pulm, renal sxs (bleeding)
Immunofluoresence

139

Myelin basic protein, proteolipid protein

Implicated in MS

140

Stony fruits

Associated with oral allergy syndrome

141

SLE treatment when steroids fail

Cyclophosphamide

142

Abatacept

CTLA4-Ig fusion protein
Used in RA

143

Mycophenolate

Inhibits IMP DH (guanine synthesis).
Transplant alternative to cyclophosphamide
Also used in AI diseases, vasculitides

144

Denosumab

Anti-rank L
used in osteoporosis

145

Basiliximab

mAb against IL-2alpha

146

Rituximab

Anti-CD20

147

Efalizumab

mAb against CD11a

148

Ustekinumab

mAb to p40 subunit IL-12, IL-23.
Psoriatic arthritis

149

Kveim test

Tests for sarcoidosis.
Not used in UK due to infection risk (involves placing sample of sarcoid pt's spleen intradermally into pt and biopsying in 4-6 weeks to see if caseating granulomas)

150

Latex fixation test

Used for rheumatoid factor

151

Increased CD50

Acute or chronic inflamm

152

HLA-DQ2

Celiac

153

HLA-B27

Ank spon

154

HLA-DR2

Goodpasture's

155

HLA-DR3

Graves, MG, SLE

156

HLA-DR4

T1DM
RA

157

Serum tryptase levels

Useful in diagnosing anaphylaxis

158

Anti-desmoglein 1,3

Pemphigus vulgaris

159

Isograft

Between twins

160

Hyperacute rejection

Minutes to hours.
Preformed Abs

161

Acute rejection

~1 week
Type IV HSR

162

Chronic rejection

Months-years

163

gp120

Envelope
Binds CXCR4 on surface CD4+ cell to gain entry

Initial step in HIV infection

164

gp41

Penetrates CD4+ wall

165

Tx for myasthenic crisis

Plasmapheresis

166

Natalizumab

mAb against alpha4 integrin (T cell migration)

167

Tocilizumab

mAb against IL-6
Castleman's, RA

168

Adalimumab

Fully humanized mAb to TNF-a

169

Speckled immunofluroescence pattern

Anti-Jo1
Anti-Sm
Anti-RNP
Anti-Ro

170

Nucleolar immunofluorescence pattern

anti-RNA polym (systemic sclerosis)

171

Peripheral immunofluorescence pattern

anti-dsDNA

172

Seronegative spondylarthropathies

Ank spon
Enteropathic arthritis
Psoriatic arthritis
Reactive arthritis

173

anti-1A-2 and anti-phogrin antibodies

Diabetes
Against tyrosine phosphatase

174

Hashimoto's antibodies

anti-thyroid peroxidase
anti-thyroglobulin

175

Kearns-Sayre syndrome

Initially eye stuff (ptosis, difficulties with eye movement, pigmentation retina), then prox muscle weakness, cardiac conduction defects, hearing loss, cerebellar ataxia
Hypoparathyroidism, primary gonadal failure, DM, hypopituitarism

176

Hirata's disease

Auto-Abs to serum insulin
Fasting hypoglycemia
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