Quiz 2- immunity Flashcards
1
Q
What is in a neutrophil granule?
A
Peroxidase, lysozyme, degradative enzymes, defensins
2
Q
How do neutrophils act to kill pathogens?
A
Phagocytosis, degranulation, or NETs
3
Q
What is the lifespan of a neutrophil?
A
Hours to a couple of days
4
Q
What is the lifespan of monocyte?
A
If you days normally, can live longer during inflammation
5
Q
Is the lifespan of a macrophage?
A
Long lifespan, can live up to years in tissues
6
Q
What kinds of things do macrophages do?
A
Respond to PAMPs/DAMPs, regulate extravasation of white blood cells, phagocytosis, tissue repair, produce inflammatory mediators, present antigens, and immunomodulation
7
Q
What types of dendritic cells are there?
A
Myeloid, plasmacytoid, Langerhans
8
Q
What do basophils do?
A
Parasite defense, allergic reaction
9
Q
What do mast cells do?
A
Parasite defense, allergic reactions
10
Q
What is the lifespan of a mast cell?
A
Long-lived cells
11
Q
What type of cell regulates vascular permeability?
A
Mast cells
12
Q
What type of cells are polymorphonuclear?
A
Eosinophils and neutrophils
13
Q
What do eosinophils do?
A
Parasite defense, allergic reactions
14
Q
What types of WBCs work in parasite defense and regulate allergic reactions?
A
Basophils, mast cells, eosinophils
15
Q
What are the clusters of differentiation for pluripotent stem cells, natural killer cells, T cells, and B cells?
A
Pluripotent stem cells - 34
NK - CD 56
T - 3, 4, 8
B - 19, 20
16
Q
What do eosinophil granules contain?
A
Histamine, peroxidase, lipase, and major basic protein
17
Q
What do basophil/mast cell granules contain?
A
Histamine, serotonin, heparin, cytokines, chemokines
18
Q
What are the hallmark signs of inflammation?
A
Heat, redness, swelling, pain, loss of function
19
Q
What type of WBC is involved in inflammation and what does it release?
A
Mast cell, releases prostaglandins, leukotrienes, and histamine
20
Q
What are the pyrogenic cytokines that are produced by macrophages?
A
TNF, IL-1, IL-6
21
Q
Examples of pattern recognition receptors
A
Mannose receptor, f-met receptor, toll-like receptors, NOD-like receptors
22
Q
TLR1/TLR2
A
Recognizes bacterial lipopeptides and GP1 of parasites
23
Q
TLR2/TLR6
A
Recognizes lipoteichoic acid of G+ bacteria and zymosan of yeasts (fungi)
24
Q
TLR3
A
Double stranded viral RNA
25
TLR4
LPS of G- bacteria
26
TLR5
Flagella of motile bacteria
27
TLR7
ssRNA of viruses
28
TLR8
ssRNA of viruses
29
TLR9
Unmethykated CpG-rich DNA of bacteria and viruses
30
What do DAMPs interact with to start inflammatory process?
PRRs
31
What types of cells are phagocytes?
Neutrophils, monocytes, and macrophages
32
What types of cells are involved in cell-mediated innate immune responses?
Neutrophils, monocytes, and macrophages act as phagocytes. Mast cells, basophils, and eosinophils defend against multicellular parasites and play a role in allergy response. Natural killer cells eliminate infected/malignant cells
33
What types of responses are involved in humoral innate immunity?
Complement system, acute phase proteins, and natural antibodies generated against antigens in GI tract
34
What does CRP do?
It is an acute phase protein that promotes phagocytosis as an opsonin
35
How does the humeral response of adaptive immunity work?
B cell receptors bind to specific antigens which causes them to undergo clonal expansion and differentiation into plasma cells that secrete antibodies
36
How does the cell-mediated response of adaptive immunity work?
T cells are activated by APCs and then undergo clonal expansion. Cytotoxic T cells kill infected host cells, helper T cells involved in B cell high affinity antibodies
37
What types of cells are APCs?
Macrophages, dendritic cells, and B cells
38
Tissue macrophage in CNS
Microglia
39
Tissue macrophage in liver
Kuppfer cells
40
Tissue macrophage in lung
Alveolar macrophages
41
Tissue macrophage in bone
Osteoclast
42
Tissue macrophage in spleen
Sinusoidal macrophages
43
Tissue macrophage in connective tissue
Histocyte
44
For recurrent sinopulmonary bacterial infections, you screen...?
Screen humoral immunity
45
For recurrent viral/fungal infections, you screen...?
Screen cellular immunity
46
For recurrent skin abscesses/fungal infections, you screen...?
Screen for phagocyte defect
47
For bacteremia or meningitis with encapsulated bacteria, you screen...?
Screen for complement deficiency
48
What does a diff test look for?
T-cell, B-cell, T/B cell defects
49
What does a DTH skin test look for?
T cell defects, only works if previously immunized
50
What does serum IgG, IgM, and IgA look for?
Humoral immunodeficiency
51
What does Ab testing to a specific Ag after immunization look for?
Humoral immunodeficiency
52
What does total hemolytic complement assay look for?
Functional status of complement system
53
What does nitroblue tetrazolium test look for?
Phagocytic disorder
54
What disease is typically associated with severe lymphopenia?
SCID
55
Paroxysmal Nocturnal Hemoglobinuria
Failure to regulate MAC formation. Unable to anchor complement regulatory proteins like DAF and CD59 to cell membrane due to lack of GPI anchor. RBCs most susceptible
56
MyD88 deficiency
Severe pyogenic infections but normal resistance to other infections. Patients lack fevers or elevated CRP/ESR w/ infection.
57
TLR3 deficiency
Increased susceptibility to HSV encephalitis
| Autosomal dominant
58
Type 1 hypersensitivity
```
Immediate reaction, (need cross-linking) pre-formed IgE antibodies bound to mast cells crosslink with antigen upon exposure. Mast cell degranulation (histamine, proteases, prostaglandins, leukotrienes, cytokines) = symptoms
IL-4 triggers B cells to class switch to IgE
```
59
Atopy
Genetic predisposition to hypersensitivity
60
Type 2 hypersensitivity
IgG/IgM directed against self tissues/antigens.
Phagocytosis via Ig opsonization (FcRgamma or CR1 receptors - ROS and lysosomal enzymes released), complement-mediated lysis, or ab-dependent cytotoxicity
Complement system activation - C3a and C5a (anaphylatoxins) induce inflammation
-- Complement-mediated cytotoxicity and antibody-dependent cellular cytotoxicity (ADCC)
61
Type 3 hypersensitivity
Antigen-antibody (IgG) complexes form (deposit in blood vessels) which activates complement system —> tissue/cell damage
Complement system activation - C3a and C5a (anaphylatoxins) induce inflammation.
Fc receptor-mediated inflammation.
Generalized: serum sickness
Localized: arthus reaction
62
Type 4 hypersensitivity
Delayed. Cell-mediated, no antibodies. Memory T-cells initiate immune response (ex: TB test). Triggers include autoimmunity, exaggerated/persistent responses to environmental antigens, and some microbial antigens. Tissue injury caused by inflammation induced by Th1 and Th17 cytokines, by macrophages, or by CD8+ killing
63
Type 1 examples
Asthma = spasmodic contraction of smooth muscle around bronchi. Can have non-immunologic stimuli like cold and exercise
Systemic: anaphylaxis - food allergen intake = massive release of vasoactive amines and cytokines from mast cells.
64
Allergen-specific immunotherapy (allergen-SIT)
curative approach to allergic diseases by administering increasing doses of allergen to induce peripheral T cell tolerance, increase thresholds for mast cell activation, and decrease IgE-mediated histamine release by mast cells
65
Type 2 examples
Antibodies stimulate TSH receptor without ligand = hyperthyroidism (Graves disease)
Antibodies inhibit binding of acetylcholine NT to ACh receptor = myasthenia gravis
66
Autoimmune hemolytic anemia
RBC membrane proteins opsonized for phagocytosis causing hemolytic anemia. Type 2 hypersensitivity.
67
Autoimmune thrombocytopenic purpura
Platelet membrane proteins opsonized for phagocytosis causing bleeding. Type 2 hypersensitivity.
68
Goodpasture's syndrome
Non-collagenous protein in basement membrane of kidney glomeruli/lung alveoli have complement-mediated inflammation or Fc receptor-mediated inflammation causing nephritis/lung hemorrhage. Type 2 hypersensitivity.
69
Grave's disease
TSH receptor stimulated by antibodies causing hyperthyroidism. Type 2 hypersensitivity.
70
Myasthenia gravis
Ach receptor inhibited by antibody which downregulates it and causes muscle weakness and paralysis. Type 2 hypersensitivity.
71
Pemphigus vulgaris
Antibody-mediated activation of proteases causes skin vesicles
72
Rheumatic fever
Antibodies produced against strep start cross-reacting with heart causing myocarditis. Type 2 hypersensitivity.
73
Systemic Lupus Erythematosus
Antibodies for DNA cause nephritis, arthritis, vasculitis. Type 3 hypersensitivity.
74
Polyarteritis nodosa
Microbial antigens cause vasculitis. Type 3 hypersensitivity.
75
Post-streptococcal glomerulonephritis
Streptococcal cell wall antigens cause nephritis. Type 3 hypersensitivity.
76
Serum sickness
```
Various protein antigens cause systemic vasculitis, nephritis, and arthritis. Type 3 hypersensitivity.
Immune complexes (ab-ag) in bloodstream are deposited which triggers complement response/macrophages and neutrophils
```
77
Arthus reaction
Various protein antigens cause cutaneous vasculitis. Type 3 hypersensitivity.
Local tissue reaction usually on skin. Ag-ab complexes form in tissues
78
DTH
Delayed-type hypersensitivity = T-cell mediated inflammatory reaction. Type 4 hypersensitivity.
79
MS
T-cells specific to myelin proteins causes demyelination in CNS and sensory motor dysfunction. Type 4 hypersensitivity.
80
RA
T-cells specific to antigens in joints causes inflammation of synovium and erosion of cartilage/bone in joints. Type 4 hypersensitivity.
81
DM I
T-cells specific to pancreatic islet antigens causes impaired glucose metabolism and vascular disease. Type 4 hypersensitivity.
82
Crohn's disease
T-cells specific to unknown intestinal microbes causes inflammation of bowel wall. Type 4 hypersensitivity.
83
Contact sensitivity
T-cells specific to modified skin proteins (ex: haptogen added) causes skin rash (ex poison ivy rxn). Type 4 hypersensitivity.
84
Chronic infections
T-cells specific to microbial proteins causes chronic inflammation (ex TB). Type 4 hypersensitivity.
85
Which enzymes act before Pro-T phase?
Gamma-chain, JAK3, ADA, PNP
86
Which enzymes act before Pre-B/Pre-T?
RAG1/RAG2, ARTEMIS
87
Which enzymes act before Immature B (Pre-BCR checkpoint)?
BTK
88
Which enzymes act before double positive T cell (Pre-TCR checkpoint)?
CD3
89
Which enzymes act before single positive T cell?
ZAP70, TAP1,2
90
Typical signs of SCID
Severe, persistent (opportunistic) infections, oral thrush, chronic diarrhea, failure to thrive
91
Adenosine Deaminase Deficiency
T-, B-, NK-
No Igs
Autosomal recessive, no live vaccines, HSCT
Accumulation of toxic doxyadenosine leads to lymphocyte apoptosis
2nd most common form of SCID
92
Purine Nucleotide Phosphorylase Deficiency
T-, B-, NK+/-
Normal Igs
Autosomal recessive, no live vaccines, HSCT
Accumulation of intracellular dGTP which is toxic to lymphocytes. Commonly associated w/ autoimmune disorders.
Very rare
93
ARTEMIS Deficiency
T-, B-, NK+
Low Igs
Autosomal recessive, no live vaccines, HSCT
Enzyme that repairs double strand breaks during VDJ recombination. Presentation - diarrhea, candidiasis, and opportunistic pneumocystis jiroveci (fungus). Risk for developing lymphomas.
94
RAG1/RAG2 Deficiency
T-, B-, NK+
No Igs
Autosomal recessive, no live vaccines, HSCT
Impaired V(D)J recombination, development of B/T cells stopped. Presentation - diarrhea, candidiasis, and opportunistic pneumocystis jiroveci.
LEAKY allows for Omenn Syndrome (partial dysfunction w/ severe erythroderma)
95
Jak3 Deficiency
T-, B+, NK-
Low IgM produced
Autosomal recessive, no live vaccines, HSCT
Janus Kinase 3 mutation (part of IL-2 receptor) - T-cell growth factor
96
Most common immunodeficiency (cell type)
B lymphocyte immunodeficiencies
97
Agammaglobulinemia
T+, B-, NK+
No Igs
X-linked OR autosomal recessive, no live vaccines, HSCT
B-cell development arrested at Pre-B due to BTK deficiency. No rearrangement in Ig heavy chains
98
Isolated IgG Subclass Deficiencies
T+, B+, NK+
Low IgG, normal IgM, IgA, IgE
Autosomal recessive, no vaccine restrictions, symptomatic treatment
Caused by "several" gene defects. Usually asymptomatic, sometimes associated w/ recurrent URIs
99
IgA Deficiency
T+, B+, NK+
NO IgA, Normal IgM, IgG
Autosomal (high in male), no vaccine restrictions, symptomatic treatment
B cells don't mature to plasma cells. 50% asymptomatic. Symptomatic show recurrent encapsulated bacterial infections. Patients develop anti-IgA antibodies which can cause anaphylactic response to IVIG transfusions
100
DiGeorge Syndrome
T-, B+, NK+
Normal Igs
Autosomal dominant, vaccines depend on T-cell count
Micro-deletion of chromosome 22 causes undeveloped thymus = progenator T cells can't develop. Frequent URIs.
Classic TRIAD- cardiac anomalies, hypocalcemia, and hypoplastic thymus
101
Hyper IgM Syndrome
T+, B+, NK+
High IgM, low IgG and IgA
X-linked (CD40L) AND autosomal (CD40), no polio vaccine, symptomatic treatment
Susceptible to bacterial infection. No class switching/somatic hypermutation = only IgM present.
102
Transient Hypogammaglobulinemia of Infancy
T+, B+/-, NK+
Normal IgM, low IgG and IgA
Polio vaccine not recommended, symptomatic treatment
Maternal IgG disappears after 6 months, IgG production delayed for up to 3 years. Susceptible to sinopulminary infections
103
Common Variable Immune Deficiency
T+, B+/-, NK+
Low IgG and IgA, normal IgM
Autosomal recessive, HSCT
Mutations in receptors for B-cell growth factor and costimulators. Defect in Ig production (like hypogammaglobulinemia). Recurrent infections (sinopulminary), autoimmune disease association, and risk for lymphoma. Dx at 20-30 y/o.
104
Common Gamma Chain Deficiency
T-, B+, NK-
Very low Igs
X-linked, no live vaccines, HSCT
Most common SCID. IL-2Rgamma (T-cell growth factor receptor) deficiency. No functional B cells since T cells can't help them. Presentation - failure to thrive, severe thrush, opportunistic infections, chronic diarrhea
105
IL-7R Alpha Chain Deficiency
T-, B+, NK+
Very low Igs
Autosomal recessive, no live vaccines, HSCT
Receptor for early T-cell development defective, no co-stimulation for B-cells.
106
Bare Lymphocyte Syndrome
CD4-, B+, NK+
Low Igs
Autosomal recessive, no live vaccines, HSCT
No MHC class II expression on professional APCs due to transcription factor defect = affects CD4 T-cells. Recurrent URI, GI, UTI
107
MHC Class I Deficiency
CD8-, B+, NK-
Normal Igs
Autosomal recessive, no restrictions of vaccines, symptomatic treatment
TAP1 mutation for peptide txfr to ER = deficient CD8+ cells = recurring viral infections
108
CD3 Complex Deficiencies
T-, B+, NK+
Low Igs
Autosomal recessive, no live vaccines, HSCT
Deficiency in subunit of CD3 coreceptor (delta, gamma, epsilon, zeta) means no T-cell activation. Causes failure to thrive, opportunistic infections, chronic diarrhea.
