Immuno Flashcards
1
Q
Allergic rhinitis
A
Loss smell, nasal itching and discharge, swollen and hyperemic nasal mucosa.
Tx with corticosteroid spray (fluticasone) +/- PO antihistamines
2
Q
Hereditary angioedema
A
Laryngeal edema, abdo sxs (pain, D&V, constip, distension), ?swelling hands and feet.
Triggered by infections, stress, menstruation, ACEI
3
Q
Acute urticaria
A
4
Q
Urticarial vasculitis
A
Ecchymoses and hyperpigmentation may occur in healing process
Biopsy for dx
5
Q
Classic urticaria reaction
A
Latex
6
Q
Which Ig is a dimer?
A
IgA
7
Q
Which Ig is most abundant?
A
IgG
8
Q
Chemotactic agents
A
IL8, LTB4, C5a, bacterial products, kallikrein
9
Q
Kostmann’s
A
Congenital neutropenia
10
Q
Examples of Type II HSR
A
Hemolytic anemias Graves Goodpastures ITP Myasthenia Gravis Type I DM
11
Q
Examples Type III HSR
A
SLE HCV-associated MPGN RA Polyarteritis nodosa Arthus reaction Serum sickness
12
Q
c-ANCA
A
Wegener’s
13
Q
p-ANCA
A
Polyarteritis nodosa
Microscopic polyangitis
14
Q
Live vaccines
A
MMR BCG Yellow fever Oral polio (Sabin) VZV
15
Q
Killed vaccines
A
Rabies
Influenza (IM)
Polio (salk)
HAV
16
Q
Subunit/congugate vaccine
A
Pneumococcal
17
Q
Congugate vaccine
A
Hib
18
Q
Recombinant vaccine
A
HBV
19
Q
Detoxified vaccine
A
Tetanus
20
Q
Inactivated bacterial preparation vaccine
A
Whole cell typhoid
21
Q
Alum
A
Agent that produces predominantly Ab response through release IL-4 which primes naive B cells
22
Q
Vaccination: active or passive immunity
A
Active
23
Q
Immune globulin: active or passive immunity
A
Passive
24
Q
Innate immunity composed of?
A
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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