DIT review - Immunology Flashcards

1
Q

What are the 3 functions of antibodies?

A

Opsonization, neutralization, complement activation

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2
Q

What holds the antibody structure together?

A

Disulfide bonds

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3
Q

What is the normal ratio of Lambda to Kappa immunoglobulin light chains?

A

K:L = 2:1

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4
Q

What is the mechanism behind antibody diversity?

A

V(D)J random recombination

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5
Q

What are the surface markers on T-cells

A

CD3, TCR, CD28, CD8 (cytotoxic), CD4 (helper), CD40L (helper)

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6
Q

What are the surface markers on B-cells

A

CD19, CD20, CD21, CD40, MHC II, B7, IgM, IgD

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7
Q

What are the surface markers on macrophages

A

CD14, CD40, MHC I, MHC II, B7

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8
Q

What are the surface markers on NK cells

A

CD16 (Binds to constant region of antibodies to antibody-dependent cell-mediated cytotoxicity - ADCC), CD56

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9
Q

Describe thymus-dependent vs. thymus-independent antigens

A

Thymus-independent antigens lack a peptide component and therefore cannot be presented by MHC to T-cells in order to create lasting immunity (e.g. LPS from gram - bacteria) Thymus-dependent antigens contain a peptide component so can be presented on MHC cells in order to create lasting immunity

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10
Q

What appears with apple green birefringence under Congo red stain?

A

Amyloid deposits

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11
Q

What disorder has anti-centromere antobodies?

A

Limited scleroderma (CREST syndrome - calcinosis, Ranyouds, Esophageal dysmotility, Sclerodactyly, Telangiectasia)

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12
Q

What disorder is associated with anti-desmoglein?

A

Pemphigus vulgaris

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13
Q

What auto-antibody is associated with bullous pemphigoid?

A

Anti-hemidesmosome

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14
Q

What antibodies are associated with SLE?

A

Antinuclear (ANA - nonspecific), anti-dsDNA, anti-Smith

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15
Q

What antibody is associated with drug-induced lupus?

A

Anti-histone

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16
Q

What disorder is associated with anti-Scl-70 (anti-DNA topoisomerase)

A

Diffuse scleroderma

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17
Q

What type of antibody is rheumatoid factor?

A

IgM against Fc portion of IgG

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18
Q

What antibodies are associated with Sjogren syndrome?

A

Anti-SSA, anti-SSB

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19
Q

What antibody is associated with polymyositis and dermatomyositis

A

Anti-Jo-1

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20
Q

What antibody is associated with mixed connective tissue disease?

A

Anti-U1 RNP (ribonucleotide protein)

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21
Q

What type of HSR is contact dermatitis?

A

HSR Type IV

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22
Q

What type of HSR is serum sickness?

A

Type III

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23
Q

What type of HSR is PPD test?

A

Type IV

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24
Q

What type of HSR is acute hemolytic transfusion reaction?

25
What type os HSR is acute rheumatic fever?
Type II
26
What type of HSR is rheumatoid arthritis?
Type III
27
What type of HSR is SLE
Type III
28
What complement factors are responsible for opsonization?
C3b
29
What complement factors make up MAC?
C5b, C6, C7, C8, C9
30
What complement factors are responsible for neutrophil chemotaxis?
C3a and C5a
31
Describe the defect in hereditary angioedema
Deficiency in C1 esterase inhibitor, leading to overactivation of complement and increase in bradykinin
32
Describe the defect in paraxoysmal nocturnal hemoglobinuria
Deficiency in GPI (protein that anchors DAF/CD55 to RBCs in order to protect from complement-mediated damage) Leads to chronic intravascular hemolysis and thrombosis (platelets are also destroyed and release their contents, inducing thrombosis)
33
Describe the different parts of the spleen and what cells are located in what parts
Red pulp: RBCs White pulp: Follicle - B-cells PALS - T-cells Marginal zone (between red and white pulp) = antigen presenting cells
34
What is the function of interferons
Interferons are anti-viral cytokines Glycoproteins synthesized by virus-infected cells that act locally on uninfected cells, "priming" them for viral defense by helping to selectively degrade viral nucleic acid and protein
35
What are the factors that are chemotactic for neutrophils?
IL-8, C5a, and Leukotriene B4
36
What is the funciton of IL-2?
Produced by T-cells in order to stimulate growth of more T-cells (helper, cytotoxic, regulatory) and NK cells
37
What is the function of TNF-a
Secreted by macrophages Mediates septic shock Causes WBC recruitment
38
What is the function of IL-8
Chemotaxis of neutrophils
39
What is the function of IFN-y
Produced by Th1 cells Stimulates macrophages to kill phagocytosed pathogens
40
What is the function of IL-10
Secreted by Th2 cells Inhibits macrophages and dendritic cells
41
What are the different HSR involved in hyperacute, acute, and chronic transplant rejection
Hyperacute - Type II (preformed antibodies) Acute - Type IV (cellular and humoral - but antibodies develop after transplant) Chronic - Type II and IV
42
What is graft-vs-host disease
Donor (foreign) bone marrow is transplanted into the patient, and then the donor cells begin to attack the host Type IV HSR Presents as: maculopapular rash, jaundice, diarrhea, hepatosplenomegaly
43
* Infliximab
* TNF-a inhibitor * Recall: TNF-a is an acute phase reactant produced by activated macrophages to mediate inflammation by accelerating neutrophil migration, and facilitate lymphocyte proliferation; TNF-a is responsible for fever, anorexia, corticotropin releasing hormone, septic shot, and cachexia * Mechanism of action: * Is a monoclonal antibody to TNF-a * Uses: * Used for autoimmune conditions due to anti-inflammatory effect * Rheumatoid arthritis, psoriasis, ankylosing spondylitis
44
* Etanercept
* TNF-a inhibitor * Recall: TNF-a is an acute phase reactant produced by activated macrophages to mediate inflammation by accelerating neutrophil migration, and facilitate lymphocyte proliferation; TNF-a is responsible for fever, anorexia, corticotropin releasing hormone, septic shot, and cachexia * Mechanism of action: * Mimics TNF-a receptor (aka it intercepts TNF-a before it can reach its target) * Uses: * Used for autoimmune conditions due to anti-inflammatory effect * Inflammatory bowel disease, rheumatoid arthritis, ankylosing spondylitis, psoriasis
45
* Cyclosporine
* Immunosuppressant * Blocks lymphocyte activation and proliferation * MOA: * Binds cyclophilin (protein within cytosol of T-cells) * Inhibits calcineurin (which stimulates IL-2) à prevention of IL-2 transcription * Uses: * Transplant rejection * Psoriasis * Rheumatoid arthritis * Toxicity * Nephrotoxicity
46
* Tacrolimus
* Immunosuppressant * Blocks lymphocyte activation and proliferation * MOA: * Binds FK506 binding protein * Inhibits calcineurin à prevention of IL-2 transcription * Uses: * Transplant rejection prophylaxis
47
Sirolumus (aka Rapamycin)
* Immunosuppressant * Blocks lymphocyte activation and proliferation * MOA: * Binds FKBp12 à inhibition of mTOR * Prevents _response_ to IL-2
48
* Dacluzimab
* Immunosuppressant * Blocks lymphocyte activation and proliferation * MOA: * Binds CD25 (IL-2 receptor) à preventing response to IL-2
49
* Azathioprine
* Immunosuppressant * Blocks lymphocyte activation and proliferation * MOA: * Precursor of 6-mercaptopurine * Blocks nucleotide synthesis, thus preventing cells division and replication * Toxicity increased by allopurinol * 6-MP is degraded by xanthine oxidase and allopurinol blocks xanthine oxidase
50
* Mycophenolate mofetil
* Immunosuppressant * Blocks lymphocyte activation and proliferation * MOA: * Inhibits IMP dehydrogenase à prevents synthesis of guanine
51
What is a major cause of SCID
SCID = defect in early stem cell differentiation Caused by adenosine deaminase deficiency (leads to increased dATP which is cytotoxic to lymphocytes)
52
What are the two immunodeficiencies that present with an absent thymic shadow?
DiGeorge SCID
53
Describe Wiskott-Aldrich syndrome
XLR immunodeficieny - due to T cells unable to reorganize actin skeleton WATER - Wiskott Aldrich, Thrombocytopenia, Eczema (especially truncal), Recurrent infections
54
What is the cause and presentation of ataxia telangiectasia
Immunodeficiency - decreased T-cells and IgA Defect in ATM gene - failure to repair DNA double strand breaks - cell cycle arrest Triad: Cerebellar defects (ataxia), Spider angiomas (telangectasia), IgA deficiency
55
Deficient enzyme in CGD
NADPH oxidase
56
Presentation of Chediak Higashi syndrome
Remember that it is a defect in protein trafficking: Recurrent infections Partial albinism Peripheral neuropathy
57
Cause and presentation of Hyper-IgE (Job syndrome)
o Deficiency in IFN-y lead to impaired neutrophil recruitment o Presentation à FATED § F – coarse Facies § A – Abscesses § T – retained primary Teeth § E – increased IgE (all other immunoglobulins are normal) § D – dermatologic problems (eczema)
58
Cause and presentation of leukocyte adhesion deficiency
o Defect in integrin (CD18) leading to impaired neutrophil migration and chemotaxis o Leads to recurrent bacterial infections and delayed separation of umbilical cord