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Immunity Lecture Flashcards

1
Q

What is the most numerous of all white blood cells

A

Neutrophils (40-87%)

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2
Q

What are neutrophils role?

A

Acute inflammatory cells

Involved in phagocytosis

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3
Q

Never Let Monkeys Eat Bananas

A 
N - Neutrophils (40-87%)
L - Lymphocytes (20-50%)
M - Monocytes (2-11%)
E - Eosinophils (0-6%)
B - Basophils (0-2%)
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4
Q

What are neutrophil markers?

A

CD45, CD11/18, CD13, CD15, CD33

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5
Q

What is the role of CD4+ T-Helper Cells

A

Activated by bind to class II MHC and secrete cytokines

Influence all other cells of immune system

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6
Q

What is the role of CD8+ Cytotoxic T-Cells

A

Activated by bind to Class I MHC

Directly kills virus-infected tumor cells

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7
Q

What are T-Cell Markers in Molecular testing

A

TCR, MHC-Peptide Antigen Complex

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8
Q

What are T-Cell markers in Flow Cytometry and Immunostains?

A

CD45, CD3, CD4, 8, 2, 5, 7

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9
Q

What is the role of B Cells?

A

Differentiate into plasma cells which secrete immunoglobulins

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10
Q

What are B Cells markers in Molecular testing?

A

BCR< immunoglobulin-subclasses IgM, IgA, IgD, IgG, IgE, Kappa and Lambda Light Chains

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11
Q

What are B cell markers in flow Cytometry

A

CD45, 19, 20 ,22, 79a

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12
Q

What are NK Cells Role

A

Lyse Tumor cells and virally infected cells

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13
Q

What are Nk Cell Markers in Molecular testing

A

KIR - inhibits NK cytolysis by recognition of self MHC

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14
Q

What are Macrophage roles

A

Antigen Presentation: MHC II

Phagocytosis - antigens w/complement

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15
Q

Macrophage markers for molecular testing

A

None

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16
Q

Macrophage markers for Flow Cytometry

A

CD45, 14, 64, 68, 163

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17
Q

Eosinophil Roles

A

Contains proinflammatory proteins involved in allergies

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18
Q

Flow Cytometry Eosinophil markers

A

CD45, 38, 123

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19
Q

Basophil roles

A

Contain proinflammatory granules involved in allergies

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20
Q

Basophil Flow Cytometry Markers

A

CD45, 38, 123

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21
Q

What HLA molecules are associated with MHC I?

A

HLA-A, B, C

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22
Q

How does MHC I molecules work?

A

They are present on all nucleated cells

Bind to peptides derived from proteins synthesized within cells and present them to CD8+ T-Cells

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23
Q

How does MHC Class I prevent lysis of a normal cell

A

By engaging KIRs on NKC

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24
Q

What HLA molecules are associated with MHC II

A

HLA-DR, DP, DQ

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25
Where are MHC II cells present and how do they work?
Only present on antigen presenting cells Binds to peptides derived from proteins outside the cells and present to CD4+
26
What HLA allele is involved in Ankylosing spondylitis?
B27
27
What is the classical complement pathways?
Initiation by interaction between IgM/IgG, and C1q
28
What is the alternate complement pathway?
Activation by bacterial cell walls, endotoxin, or venom binding to C3b absent of Ig C3 --> C5 --> Membrane Attack C3--> Opsonization, phagocytosis, B-lymphocyte activation
29
What cytokines mediate innate immunity?
IL-1, TNF, IL-6, interferons Macrophages are major source
30
What cytokines activate inflammatory cells
IFN-y, TNF Derived from T-Cells
31
What cytokines act to recruit inflammatory cells to sites of injury?
IL-8 Produced by macrophages and injured cells
32
What cytokines regulate lymphocyte response?
IL-2, IL-4, IL-5, IL-12
33
What cytokines stimulate hematopoiesis?
Colony Stimulating Factors (CSF)
34
What 2 global tests are used for the immune system?
WBC Count with differentials Peripheral Smear Examination
35
How is B-Cell function tested?
Ig levels and isotope titer Flow Cytometry
36
How is T-Cell function tested?
Delayed type hypersensitivity testing Flow cytometry
37
What are the key uses for flow cytometry?
Blood cell population analysis, Cancer diagnostics, cell sorting
38
What are the 2 steps in interpreting flow cytometry dot plots?
1. Establish clonality of B-Cells | 2. Subclassify the disease
39
How do you test neutrophil function?
Neutrophil count and smear MPO Staining Nitroblue tetrazolium (NBT)
40
Define hypersensitivity reactions
Excessive Immune responses that cause tissue injury and diseases Dependent on adaptive immune system
41
What is required in hypersensitivity reactions to prime the adaptive immune response?
Prior Antigen exposure
42
What are causes of Hypersensitivity reactions
Failure of self-tolerance to self Uncontrolled responses against foreign antigens
43
What is Type 1 hypersensitivity?
Allergies - IgE
44
What is Type 2 Hypersensitivity
Antibody-mediated - IgG, IgM phagocytosis and lysis of cells Autoimmune disease
45
What is Type 3 Hypersensitivity
Immune Complex Mediated - Ag-Ab inflammation, necrotizing vasculitis Systemic lupus erthematosus, glomerulonephritis, serum sickness
46
What is Type 4 Hypersensitivity
Cell Mediated - T-Lymphocytes Perivascular cellular infiltrates, edema Contact dermatitis, multiple sclerosis
47
What does ACID stand for?
Anaphylactic Cytotoxic Immune Complex Delayed-Type Hypersensitivity
48
What are primary immunodeficiency disease
BCS, TC, X-Linked WAS
49
What are secondary immunodeficiency disease
Infection, malnutrition, aging, chemoradiation, immunosuppression, autoimmunity
50
What are manifestations Defect in Ig, Completement or phagocytic cells
Recurrent pyogenic bacterial infections
51
What are manifestations of Cell-mediated Immunity
Viral, Fungal, and intracellular bacterial infections
52
What are 4 B Cell Defects?
B- Bruton (x-linked) agammaglobulinemia C- Common Variable Immunodeficiency (CVID) S - Selective IgA Deficiency H - Hyper IgM syndrome
53
What are T cell Defects (2)
Thymic Hypoplasia - Digeorge Syndrome Chronic mucocutaneous candidiasis
54
What are Combined B & T Cell Defects?
X-linked WAS --> X-linked lymphoproliferative disorder WAS Ataxia Telangiectasia Severe Combined Immunodeficiency
55
Bruton (X-linked) Agammaglobulinemia Characteristics
Prevalence: 1/100K Gene Defect (Xq21.3022): Mutant Bruton's tyrosine kinase involved in pre-B cell signal transduction B-Cell maturation stops after the initial heavy chain rearrangement: light chains not produced
56
What age does Bruton (X-linked) Agammaglobulinemia manifest
6 months of age as maternal immunoglobulin depletes
57
How do infections occur in Bruton (X-linked) Agammaglobulinemia
By encapsulated bacteria, cleared by antibody opsonization Susceptible to certain viral infections and guard Prone to autoimmune disease
58
What are the characteristics of peripheral blood in Bruton (X-linked) Agammaglobulinemia
Absent or decreased mature B-Cells and depressed serum levels of all Ig classes
59
What are the characteristics of lymph nodes in Bruton (X-linked) Agammaglobulinemia
Absent/Rudimentary germinal centers and absence of plasma cells
60
What are the characters if the GI tract in Bruton (X-linked) Agammaglobulinemia
Absence of plasma cells and possible giardia infection
61
What is the treatment for Bruton (X-linked) Agammaglobulinemia
IVIg, antibiotics, gene therapy
62
What is the prevalence, gene defects and general characteristics of CVID?
Prevalence: 1/50K Gene Defect: Heterogenous (ICOS, TACI) - leads to insufficient activation to Ig plasma cells Normal B cells - absent plasma Low IgG, IgA, IgM
63
What is the onset of CVID
15-35 years in both M and F
64
What are symptoms/signs of CVID
Impaired antibody response to infection: Pneumonia, Sinusitis, GI infection Weakened T Cell Immunity lymphoid Malignancy
65
Treatment for CVID
IVIg
66
What is the prevalence and gene defects of Selective IgA deficiency
Most Common primary immunodeficiency: 1/700 Defect: Intrinsic B Cell Defects or altered T-Cell Cytokine Production Low IgA
67
What is the presentation of selective IgA deficiency?
Recurrent Sinopulmonary infections, diarrhea, autoimmune diseases, anaphylaxis during blood transfusion
68
Hyper-IgM Syndrome Characteristics
Failure to isotype switch 70% due to defective CD154 on x chromosome Low levels of opsonizing IgG Increased susceptibility to pathogens that require intact DTH responses
69
What are the gene defects in DiGeorge Syndrome
90% due to 22q11 depletion Malformation in 3rd and 4th pharyngeal pouches - form thymus Absent T-Cells
70
What is the presentation of DiGeorge Syndrome?
``` Catch-22: C- Cardiac Defect A - Abnormal Facies T - Thymic Hypoplasia C - Cleft Palate H - HypoCa2+ ```
71
Characteristics of Chronic Mucocutaneous Candidiasis
Selective T-Cell Immunodeficiency against Cadida Species Presents: Chronic, Recalcitrant mucocutaneous candidal infections Does not represent specific disease, but a phenotypic spectrum
72
X-Linked Lymphoproliferative Disease Gene Defects
Xq25 mutation --> Deletion in the SH2D1A gene which codes for SH2 domain on a SAP protein
73
What does a deletion in SH2D1A gene in X-Linked Lymphoproliferative Disease cause?
leads to lack of modulation of IFN-y, causing uncontrolled cell proliferation
74
What is the presentation of X-Linked Lymphoproliferative Disease
Overwhelming T-cell mediated response to EBV Death from bone marrow failure Irreversible hepatitis Malignant Lymphoma
75
WAS gene defects
Wiskott-Adrich Syndrome X-linked recessive mutation of WASP - responsible for cytoskeletal malleability Responsible for intracellular signaling
76
Presentation of WAS
WIPE: W - WAS I - Immunodeficiency: progressive age-related depletion of T-Cells, decreased IgM P - Purpura E - Eczema Lymphoid Malignancy

Pathology Exam 3 (4 decks)

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