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Immunology/Pathology Flashcards

1
Q

What is a primary follicle of the lymph node? Secondary follicle?

A

Primary follicles are dense and dormant. Secondary have pale central germinal centers and are active.

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2
Q

What is within the paracortex of the lymph node?

A

T cells

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3
Q

HLA A3 is associated with what disease(s)?

A

Hemochromatosis

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4
Q

HLA B27 is associated with what disease(s)?

A

Psoriatic arthritis, akylosing spondylitis, arthritis of inflammatory bowel disease, reactive arthritis (formerly Reiter syndrome)

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5
Q

HLA DQ2/DQ8 is associated with what disease(s)?

A

Celiac

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6
Q

HLA DR2 is associated with what disease(s)?

A

Multiple sclerosis, hay fever, SLE, Goodpasture

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7
Q

HLA DR3 is associated with what disease(s)?

A

DM type 1, SLE, Graves disease

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8
Q

HLA DR4 is associated with what disease(s)?

A

Rheumatoid arthritis, DM type 1

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9
Q

HLA DR5 is associated with what disease(s)?

A

Pernicious anemia –> VitB12 deficiency, Hashimoto thyroiditis

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10
Q

What secretes IL-1 and what is its function?

A

Macrophage; Aka osteoclast-activating factor, an endogenous pyrogen that causes fever and acute inflammation. Activates endothelium to express adhesion molecules; induces chemokine secretion to recruit leukocytes.

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11
Q

What secretes IL-6 and what is its function?

A

Macrophage and Th2 cells; Endogenous pyrogen. Causes fever and stimulates production of acute-phase proteins.

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12
Q

What secretes IL-8 and what is its function?

A

Macrophage; Major chemotactic factor for neutrophils

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13
Q

What secretes IL-12 and what is its function?

A

Macrophage and B cell; Induces differentiation of T cells into Th1 cells. Activates NK cells.

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14
Q

What secretes TNF-alpha and what is its function?

A

Macrophage; Mediates septic shock. Activates endothelium. Causes leukocyte recruitment, vascular leak.

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15
Q

What secretes IL-2 and what is its function?

A

All T cells; Stimulates growth of helper, cytotoxic, and regulatory T cells

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16
Q

What secretes IL-3 and what is its function?

A

All T cells; Supports the growth and differentiation of bone marrow stem cells. Functions like GM-CSF.

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17
Q

What secretes interferon-gamma and what is its function?

A

Th1 cells; Has antiviral and antitumor properties Activates NK cells to kill virus-infected cells. Increases MHC expression and antigen presentation in all cells.

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18
Q

What secretes IL-4 and what is its function?

A

Th2 cells; Induces differentiation into Th2 cells. Inhibit Th1 cells. Promotes growth of B cells. Enhances class switching to IgE and igG.

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19
Q

What secretes IL-5 and what is its function?

A

Th2 cells; Promotes differentiation of B cells. Enhances class switching to IgA. Stimulates the growth and differentiation of eosinophils.

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20
Q

What secretes IL-10 and what is its function?

A

Th2, regulatory T cells, and macrophages; Modulates inflammatory response. Inhibits actions of activated T cells and Th1. Macrophages secrete it to inhibit inflammatory response.

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21
Q

What are the cell surface proteins of T cells?

A

MHC I, TCR (binds antigen-MHC complex), CD3 (associated with TCR for signal transduction), CD28 (binds B7 on APC)

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22
Q

What are the cell surface proteins of Helper T cells?

A

CD4, CD40 ligand

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23
Q

What are the cell surface proteins of Cytotoxic T cells?

A

CD8

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24
Q

What are the cell surface proteins of B cells?

A

Ig (binds antigen), CD19, CD20, CD21 (receptor on EBV), CD40, MHC II, B7

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25
Q

What are the cell surface proteins of macrophages?

A

CD14 (a TLR that recognizes LPS), CD40, MHC II, B7, Fc and C3b receptors (enhanced phagocytosis)

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26
Q

What are the cell surface proteins of NK cells?

A

CD16 (binds Fc of IgG), CD56 (unique marker for NK)

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27
Q

What is NF-kappaB and what does it do?

A

A transcription factor that is turned on as a result of TLR activation. It activates immune response genes and leads to production of multiple immune mediators.

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28
Q

What is the role of arachidonic acid in inflammation?

A

Acted on by COX of 5-lipooxygenase,

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29
Q

What released arachidonic acid?

A

Phospholipase A2

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30
Q

What is the role of MHC?

A

Present antigen fragments to T cells and bind TCRs

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31
Q

MHC II is expressed where?

A

Only on APCs

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32
Q

What is the specific function of MHC I?

A

Present endogenously synthesized antigens to CD8 cytotoxic T cells

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33
Q

What is the specific function of MHC II?

A

Present exogenously synthesized proteins to T-helper cells

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34
Q

What do NK cells do?

A

Use perforin and granzymes to induce apoptosis of virally infected cells and tumor cells.

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35
Q

What is the role of CD4 T cells?

A

Help B cells make Ab and produce cytokines to activate other cells of immune system

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36
Q

What is the role of CD8 T cells?

A

Kill virus-infected cells directly

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37
Q

Which cells are responsible for delayed cell-mediated hypersensitivity (type IV)?

A

CD8 T cells

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38
Q

What does IFN-gamma do?

A

Differentiates helper T cells into Th1 cells, activates macrophages

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39
Q

What is the role of TGF-beta + IL-6?

A

Helper T cell –> Th17 cell

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40
Q

What is the role of TGF-beta?

A

Helper T cell –> Treg cell, angiogenesis, fibrosis, cell cycle arrest

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41
Q

What are three APCs?

A

B cells, macrophages, dendritic cells

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42
Q

The interaction between a naive T cell and an APC involves which receptors?

A

MHC I/II with presenting antigen bind to TCR on CD8/4 T cells. There is a costimulatory interaction of B7 (APC) and CD28 (T cell)

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43
Q

Describe how B cells are activated?

A

Helper T cells are first activated. B cell (APC) endocytoses antigen and presents it on MHC II which is recognized by TCR on Th cell. CD40 receptor on B cells binds to CD40 ligand on Th cell (costimulatory). Th cell secretes cytokines that determine Ig class switching of B cell. B cell activates and undergoes class switching, affinity maturation, and antibody production.

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44
Q

What does the Th1 cell secrete and what does it do?

A

IFN-gamma; activates macrophages and cytotoxic T lymphocytes

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45
Q

What is the Th1 cell inhibited by?

A

IL-4 and IL-10 from Th2 cell

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46
Q

What does the Th2 cell secrete and what does it do?

A

IL-4, IL-5, IL-6, IL-13; recruits eosinophils for parasite defense and promotes IgE production by B cells

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47
Q

What is the Th2 cell inhibited by?

A

IFN-gamma from Th1 cell

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48
Q

What are the cell surface markers of regulatory T cells?

A

CD3, CD4, CD25, and transcription factor FOXP3

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49
Q

What do activated regulatory T cells produce?

A

Anti-inflammatory cytokines like IL-10 and TGF-beta

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50
Q

What is a thymus-independent antigen?

A

Antigen lacking a peptide component; cannot be presented by MHC to T cells. Weakly or nonimmunogenic.

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51
Q

What is a thymus-dependent antigen?

A

Antigen containing a protein component. Class switching and immunologic memory occur as a result of direct contact of B cells with Th cells (CD40-CD40 ligant interaction)

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52
Q

What are acute-phase reactants?

A

Factors whose serum concentrations change significantly in response to inflammation; produced by the liver in both acute and chronic inflammatory states.

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53
Q

What induces acute-phase reactants?

A

IL-6, IL-1, TNF-alpha, and IFN-gamma

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54
Q

What does hepcidin do?

A

Prevents release of iron bound by ferritin –> anemia of chronic disease

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55
Q

Is albumin upregulated or downregulated in inflammation? Why?

A

Downregulated - to conserve amino acids for production of positive reactants

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56
Q

What is the classic pathway of complement activation?

A

IgG or IgM mediated

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57
Q

What is the alternative pathway of complement activation?

A

Microbe surface molecule mediated

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58
Q

What is the lectin pathway of complement activation?

A

Mannose or other sugars on microbe surface responsible for activation

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59
Q

What is the function of C3b?

A

Opsonization

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60
Q

What is the function of C3a, C4a, and C5a?

A

Anaphylaxis

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61
Q

What is the function of C5a?

A

Neutrophil chemotaxis

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62
Q

What is the function of C5b-C9?

A

Cytolysis by membrane attack complex (MAC) - responsible for defense against gram neg bacteria

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63
Q

What are the two primary opsonins in bacterial defense?

A

C3b and IgG

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64
Q

What helps prevent complement activation of self cells?

A

Decay-accelerating factor (DAF, aka CD55) and C1 esterase inhibitor

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65
Q

C1 esterase inhibitor deficiency causes what?

A

Hereditary angioedema

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66
Q

C3 deficiency causes what?

A

Increases risk of severe, recurrent pyogenic sinus and respiratory tract infections; increased susceptibility to type III hypersensitivity reactions

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67
Q

DAF (GPI anchored enzyme) deficiency causes what?

A

Complement-mediated lysis of RBCs and paroxysmal nocturnal hemoglobinuria

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68
Q

What is the role of IF-alpha and beta?

A

Innate host defense against RNA and DNA viruses; Synthesized by viral-infected cells that act locally on uninfected cells, priming them for viral defense. “Warning and suicide”

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69
Q

What do superantigens do to the immune system?

A

Cross-link the beta region of the T cell R to the MHC class II on APCs. Can activate any T cell, leading to massive release of cytokines.

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70
Q

How do endotoxins (LPS) interact with the immune system?

A

Directly stimulate macrophages by binding to endotoxin receptor CD14; Th cells not involved

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71
Q

Does a live attenuated vaccine usually induce a cellular or humoral response?

A

Cellular

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72
Q

Does an inactivated or killed vaccine usually induce a cellular or humoral response?

A

Humoral

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73
Q

An anti-ACh receptor autoantibody is associated with what disorder?

A

Myasthenia gravis

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74
Q

An anti-basement membrane autoantibody is associated with what disorder?

A

Goodpasture

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75
Q

An anti-cardiolipin, lupus anticoagulant autoantibody is associated with what disorder?

A

SLE, antiphospholipid syndrome

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76
Q

An anticentromere autoantibody is associated with what disorder?

A

Limited scleroderma (CREST syndrome)

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77
Q

An anti-desmoglein autoantibody is associated with what disorder?

A

Pemphigus vulgaris

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78
Q

An anti-dsDNA, anti-Smith autoantibody is associated with what disorder?

A

SLE

79
Q

An anti-glutamate decarboxylase autoantibody is associated with what disorder?

A

Type 1 DM

80
Q

An anti-hemidesmosome autoantibody is associated with what disorder?

A

Bullous pemphigoid

81
Q

An antihistone autoantibody is associated with what disorder?

A

Drug-induced lupus

82
Q

An anti-Jo-1, anti-SRP, anti-Mi-2 autoantibody is associated with what disorder?

A

Polymyositis, dermatomyositis

83
Q

An antimicrosomal, antithyroglobulin autoantibody is associated with what disorder?

A

Hashimoto thyroiditis

84
Q

An antimitochondrial autoantibody is associated with what disorder?

A

Primary biliary cirrhosis

85
Q

An antinuclear antibody autoantibody is associated with what disorder?

A

SLE, nonspecific

86
Q

An anti-Scl-70 (anti-DNA topoisomerase I) autoantibody is associated with what disorder?

A

Scleroderma (diffuse)

87
Q

An anti-smooth muscle autoantibody is associated with what disorder?

A

Autoimmune hepatitis

88
Q

An anti-SSA, anti-SSB (anti-Ro, anti-La) autoantibody is associated with what disorder?

A

Sjogren syndrome

89
Q

An anti-TSH autoantibody is associated with what disorder?

A

Graves disease

90
Q

An anti-U1 RNP (ribonucleoprotein) autoantibody is associated with what disorder?

A

Mixed connective tissue disease

91
Q

A c-ANCA autoantibody is associated with what disorder?

A

Granulomatosis with polyangiitis (Wegener)

92
Q

An IgA antiendomysial, IgA anti-tissue transglutaminase autoantibody is associated with what disorder?

A

Celiac disease

93
Q

A p-ANCA autoantibody is associated with what disorder?

A

Microscopic polyangiitis, Churg-Strauss syndrome

94
Q

An rheumatoid factor, anti-CCP autoantibody is associated with what disorder?

A

Rheumatoid arthritis

95
Q

An immunocompromised patient with no T cell function will get what kind of bacterial infection(s)?

A

Sepsis

96
Q

An immunocompromised patient with no B cell function will get what kind of bacterial infection(s)?

A

Encapsulated bacteria

97
Q

An immunocompromised patient with no granulocyte function will get what kind of bacterial infection(s)?

A

Staph, Serratia, Nocardia

98
Q

An immunocompromised patient with no complement function will get what kind of bacterial infection(s)?

A

Neisseria

99
Q

An immunocompromised patient with no T cell function will get what kind of viral infection(s)?

A

CMV, EBV, JCV, VZV chronic infection with respiratory/GI viruses

100
Q

An immunocompromised patient with no B cell function will get what kind of viral infection(s)?

A

Enteroviral encephalitis, poliovirus

101
Q

An immunocompromised patient with no T cell function will get what kind of fungal/parasitic infection(s)?

A

Candida, PCP

102
Q

An immunocompromised patient with no B cell function will get what kind of fungal/parasitic infection(s)?

A

GI giardiasis (no IgA)

103
Q

An immunocompromised patient with no granulocyte function will get what kind of fungal/parasitic infection(s)?

A

Candida, Aspergillus

104
Q

Describe a Type I hypersensitivity reaction

A

Anaphylactic and atopic - Free antigen cross links IgE on presensitized mast cells and basophils. Triggers immediate release of vasoactive amines that act at postcapillary venules.

105
Q

Describe a Type II hypersensitivity reaction

A

Cytotoxic (antibody-mediated) - IgM and IgG bind to fixed antigen on “enemy” cell, leading to cellular destruction.

106
Q

Describe a Type III hypersensitivity reaction

A

Antigen-antibody complexes activate complement, which attracts neutrophils; neutrophils release lysosomal enzymes

107
Q

Describe a Type IV hypersensitivity reaction

A

Delayed (T-cell mediated) type - sensitized T cells encounter antigen and then release lymphokines (leads to macrophage activation; no Ab involved)

108
Q

What is a direct Coombs’ test?

A

Detects Abs that have adhered to patient’s RBCs

109
Q

What is an indirect Coombs’ test?

A

Detects Abs that can adhere to other RBCs

110
Q

What are the three mechanisms of Type II hypersensitivity reaction?

A
  1. Opsonization leading to phagocytosis or complement activation
  2. Complement-mediated lysis
  3. Antibody-dependent cell-mediated cytotoxicity, usu due to NK cells or macrophages
111
Q

What type of hypersensitivity reaction is “serum sickness?” And describe what serum sickness is.

A

An immune-complex disease (Type III) in which Abs to foreign proteins are produced (takes 5 days). Complexes form and are deposited in membranes, where they fix complement. Mostly caused by drugs.

112
Q

What type of hypersensitivity reaction is “arthus reaction?” And describe what arthus reaction is.

A

A local subacute Ab-mediated hypersensitivity (Type III) reaction. Intradermal injection of antigen induces Abs, whcih form Ag-Ab complexes in the skin. Characterized by edema, necrosis, and activation of complement.

113
Q

What type of hypersensitivity reaction is Guillain-Barre?

A

IV

114
Q

What type of hypersensitivity reaction is Pemphigus vulgaris and bullous pemphigoid?

A

II

115
Q

What type of hypersensitivity reaction is Polyarteritis nodosa?

A

III

116
Q

What type of hypersensitivity reaction is Idiopathic thrombocytopenic purpura?

A

II

117
Q

What type of hypersensitivity reaction is Multiple sclerosis?

A

IV

118
Q

What type of hypersensitivity reaction is Erythroblastosis fetalis?

A

II

119
Q

What type of hypersensitivity reaction is SLE?

A

III

120
Q

What type of hypersensitivity reaction is Contact dermatits?

A

IV

121
Q

What type of hypersensitivity reaction is acute hemolytic transfusion reaction?

A

II

122
Q

Describe X-linked (Bruton) agammaglobulinemia

A

Defect in BTK, a tyrosine kinase gene –> no B cell maturation

123
Q

What is the clinical presentation of Bruton agammaglobulinemia?

A

Recurrent bacterial and enteroviral infections after 6 months (when maternal IgG wears off), and giardia

124
Q

What are the findings of Bruton agammaglobulinemia?

A

Normal CD19 B cell count, decreased pro-B, decreased IgG of all classes. Absent/scanty lymph nodes and tonsils.

125
Q

Describe Selective IgA deficiency

A

Uknown etiology. Most common primary immunodeficiency. Forms anti-IgA IgGs - autoimmune. Anaphylactic reactions.

126
Q

What is the clinical presentation of IgA deficiency?

A

Usu asymptomatic. Can see airway and GI infections.

127
Q

What are the findings of IgA deficiency?

A

IgA

128
Q

Describe common variable immunodeficiency

A

Defect in B-cell differentiation. Many causes.

129
Q

What is the clinical presentation of common variable immunodeficiency?

A

Can be acquired in 20s-30s. Increased risk of autoimmune disease. Bronchiectasis, lymphoma, sinopulmonary infections.

130
Q

What are the findings of common variable immunodeficiency?

A

Low plasma cells and low immunoglobulins

131
Q

Describe Thymic aplasia (DiGeorge syndrome)

A

22q11 deletion; failure to develop 3rd and 4th pharyngeal pouches. Absent thymus and parathyroids.

132
Q

What is the clinical presentation of Thymic aplasia (DiGeorge syndrome)?

A

Tetany (hypocalcemia), recurrent viral/fungal infections, conotruncal abnormalities

133
Q

What are the findings of Thymic aplasia (DiGeorge syndrome)?

A

Low T cells, low PTH, hypocalcemia, absent thymus, 22q11 deletion detected by FISH

134
Q

Describe IL-12 receptor deficiency

A

Decreased Th1 response. Aut rec.

135
Q

What is the clinical presentation of IL-12 receptor deficiency?

A

Disseminated mycobacterial and fungal infections; may present after admin of BCG vaccine

136
Q

What are the findings of IL-12 receptor deficiency?

A

Decreased IFN-gamma

137
Q

Describe Autosomal dominant hyper-IgE syndrome (Job syndrome)

A

Deficiency of Th17 cells due to STAT3 mutation –> impaired recruitment of neutrophils to sites of infection

138
Q

What is the clinical presentation of Autosomal dominant hyper-IgE syndrome (Job syndrome)?

A

“FATED” - course Facies, cold (non-inflamed) staph Abscesses, retained primary Teeth, increased IgE, Dermatologic problems (eczema)

139
Q

What are the findings of Autosomal dominant hyper-IgE syndrome (Job syndrome)?

A

High IgE, low IFN-gamma

140
Q

Describe Chronic mucocutaneous candidiasis

A

T cell dysfunction. Many causes.

141
Q

What is the clinical presentation of mucocutaneous candidiasis?

A

Noninvasive Candida albicans infections of skin and mucous membranes

142
Q

What are the findings of mucocutaneous candidiasis?

A

Absent in vitro T cell proliferation in response to Candida antigens. Absent cutaneous reaction to Candida antigens.

143
Q

Describe SCID

A

Several types including defective IL-2R gamma chain (most common, X linked), adenosine deaminase deficiency (aut rec)

144
Q

What is the clinical presentation of SCID?

A

Failure to thrive, chronic diarrhea, thrush. Recurrent viral, bacterial, fungal, and protozoal infections.

145
Q

What are the findings of SCID?

A

Low T cell receptor excision circles. Absent thymic shadow, germinal centers, and T cells.

146
Q

Describe Ataxia-telangiectasia

A

Defects in ATM gene –> DNA ds breaks –> cell cycle arrest

147
Q

What is the clinical presentation of Ataxia-telangiectasia?

A

Triad: cerebellar defects (Ataxia), spider Angiomas (telangiectasia), IgA deficiency

148
Q

What are the findings of Ataxia-telangiectasia?

A

Increased AFP, decreased IgA IgG and IgE. Lymphopenia, cerebellar atrophy.

149
Q

Describe Hyper-IgM syndrome

A

Most commonly due to defective CD40L on Th cells = class switching defect; X linked recessive

150
Q

What is the clinical presentation of Hyper-IgM syndrome?

A

Severe pyogenic infections early in life; opportunistic infection with PCP and CMV.

151
Q

What are the findings of Hyper-IgM syndrome?

A

Increased IgM, and decreased IgG IgA and IgE

152
Q

Describe Wiskott-Aldrich syndrome

A

Mutation in WAS gene (X recessive); T cells unable to reorganize actin cytoskeleton

153
Q

What are the findings of Wiskott-Aldrich syndrome?

A

Decreased to normal IgG and IgM. Increased IgE and IgA. Fewer and smaller platelets.

154
Q

What is the clinical presentation of Wiskott-Aldrich syndrome?

A

“WATER” - Wiskott-Aldrich: Thrombocytopenia purpura, Eczema, Recurrent infections. Increased risk of autoimmune disease and malignancy.

155
Q

Describe Leukocyte adhesion deficiency (type 1)

A

Defect in LFA-1 integrin (CD18) protein on phagocytes; impaired migration and chemotaxis; aut rec.

156
Q

What is the clinical presentation of Leukocyte adhesion deficiency (type 1)?

A

Recurrent bacterial skin and mucosal infections, absent pus formation, impaired wound healing, delayed separation of umbilical cord (>30days)

157
Q

What are the findings of Leukocyte adhesion deficiency (type 1)?

A

Increased neutrophils. Absence of neutrophils at infection sites.

158
Q

Describe Chediak-Higashi syndrome

A

Defect in lysosomal trafficking regulator gene (LYST). Microtubule dysfunction in phagosome-lysosome fusion; aut rec.

159
Q

What is the clinical presentation of Chediak-Higashi syndrome?

A

Recurrent pyogenic infections by staph and strep, partial albinism, peripheral neuropathy, progressive neurodegeneration infiltrative lymphohistiocytosis

160
Q

What are the findings of Chediak-Higashi syndrome?

A

Giant granules in neutrophils and platelets. Pancytopenia. Mild coagulation defects.

161
Q

Describe Chronic granulomatous disease

A

Defect of NADPH oxidase. Decreased ROS and absent respiratory burst in neutrophils; X linked recessive.

162
Q

What is the clinical presentation of Chronic granulomatous disease?

A

Increased susceptibility to catalase positive organisms

163
Q

What are the findings of Chronic granulomatous disease?

A

Abnormal dihydrorhodamine (flow cytometry) test. Nitroblue tetrazolium dye reduction test is negative.

164
Q

What is the treatment of SCID?

A

Bone marrow transplant (no concern for rejection)

165
Q

What is the pathogenesis of hyperacute transplant rejection? (within minutes)

A

Pre-existing recipient Abs react to donor antigen (type II reaction), activate complement. Leads to widespread thrombosis of graft vessels.

166
Q

What is the pathogenesis of acute transplant rejection? (weeks to months)

A

Cellular: CTLs activated against donor MHCs. Humoral: Similar to hyperacute, except Abs develop after transplant. Results in vasculitis of graft vessels with dense interstitial lymphocytic infiltrate. Prevent/reverse with immunosuppressants.

167
Q

What is the pathogenesis of chronic transplant rejection? (months to years)

A

Recipient T cells perceive donor MHC as recipient MHC and react against donor antigens presented. Both cellular and humoral.

168
Q

What is the pathogenesis of graft-versus-host transplant rejection? (timeline varies)

A

Grafted immunocompetent T cells proliferate in the immunocompromised host and reject host cells with “foreign” proteins –> severe organ dysfunction. Presents as maculopapular rash, jaundice, diarrhea, hepatosplenomegaly. Usu in bone marrow and liver transplants.

169
Q

How does chronic transplant rejection manifest in the heart?

A

Atherosclerosis

170
Q

How does chronic transplant rejection manifest in the lungs?

A

Bronchiolitis obliterans

171
Q

How does chronic transplant rejection manifest in the liver?

A

Vanishing bile ducts

172
Q

How does chronic transplant rejection manifest in the kidney?

A

Vascular fibrosis, glomerulopathy

173
Q

When may graft-vs-host be beneficial?

A

In bone marrow transplant for leukemia (graft-vs-tumor effect)

174
Q

Which four things attract neutrophils?

A

C5a, IL-8, LTB4, bacterial products

175
Q

What is the mechanism through which inflammation causes fever?

A

Macrophage releases IL-1 and TNF which travel through bloodstream to perivascular cells of hypothalamus. This activates the COX pathway to increase PGE2 which raises temperature set point in the hypothalamus.

176
Q

In which vessels does leukocyte extravasation occur?

A

Postcapillary venules

177
Q

How do CD8 T cells kill?

A

Release cytotoxic granules containing preformed proteins (perforin - helps to deliver content of granules into target cell; granzyme B - a serine protease that activates apoptosis in target cell; granulysin - antimicrobial, induces apoptosis)

178
Q

What mediates B cell isotype switching?

A

IL-4 and IL-5

179
Q

What is the defining cell of a granuloma?

A

Epithelioid histiocyte (macrophage with abundant pink cytoplasm)

180
Q

What are the steps of granuloma formation?

A

A macrophage presents an antigen via MHC II to CD4 helper T cell. Macrophage secretes IL-12 inducing differentiation of CD4 cell to Th1 subtype. Th1 secretes IFN-gamma which causes macrophages to become epithelioid histiocytes.

181
Q

What is the difference between branchial arches and pharyngeal arches?

A

None! The branchial apparatus is also called the pharyngeal apparatus. This was just confusing me..

182
Q

A defective CD40 or CD40ligand causes what disease?

A

Hyper-IgM syndrome

183
Q

Lynch syndrome is a defect in which genes?

A

MSH2, MLH1, MSH6, or PMS2

184
Q

What are the associated neoplasms of Lynch syndrome?

A

Colorectal cancer, endometrial cancer, ovarian cancer

185
Q

What is the mutated gene in Familial adenomatous polyposis?

A

APC

186
Q

What are the associated neoplasms of familial adenomatous polyposis?

A

Colorectal cancer, desmoids and osteomas, brain tumors

187
Q

What are the associated neoplasm of von Hippel-Lindau syndrome?

A

Hemangioblastomas, clear cell renal carcinoma, pheochromocytoma

188
Q

What is the mutated gene in Li-Fraumeni syndrome?

A

TP53

189
Q

What are the associated neoplasms of Li-Fraumeni syndrome?

A

Sarcomas, breast cancers, brain tumors, adrenocortical carcinoma, leukemia

190
Q

What are the associated neoplasms of multiple endocrine neoplasia type 1?

A

Parathyroid adenomas, pituitary adenomas, pancreatic adenomas

191
Q

What is the mutated gene is multiple endocrine neoplasia type 1?

A

MEN1

192
Q

What is the mutated gene in multiple endocrine neoplasia type 2?

A

RET, MEN2A

193
Q

What are the associated neoplasms of multiple endocrine neoplasia type 2?

A

Medullary thyroid cancer, pheochromocytoma, parathyroid hyperplasia (MEN2A)

194
Q

What is the pathogenesis of multiple endocrine neoplasia type 2?

A

Aut dom, activating gain-of-function mutation in proto-oncogene, continuous stimulation of cell division predisposes to tumor growth

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