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Robbins Pathology MOD1 (Chptr 1-6) > Chapter 6-Immuno and Hypersensitivity > Flashcards

Chapter 6-Immuno and Hypersensitivity Flashcards

1
Q

What are Langerhans cells

A

Immature dendritic cells in the epidermis

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2
Q

What is the high affinity receptor on Mast cells and basophils

A

FcER1

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3
Q

How many days after antigen exposure does inflammation occur in type 3 hypersensitivity

A

10

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4
Q

What are the characteristics of the Ig alpha (CD79 alpha and beta) and beta in the BCR complex

A

Invariant proteins responsible for the signal transduction into the cell

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5
Q

What are drugs that can activate mast cells

A

Codeine, morphine, adenosine, melittin (in bee venom)

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6
Q

What are the characteristics of the autoimmune diseases

A

Chronic, with relapses and remissions, and damage is progressive

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7
Q

Innate immune receptors target what general characteristics of PAMPS on microbes

A

Microbial components that are essential for infectivity and thus can not be mutated to evade recognition

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8
Q

What disease is associated with HLA-B27

A

Ankylosis spondylitis

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9
Q

Many autoimmune diseases are associated with which HLA alleles

A

Class 2

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10
Q

What percentage of cells do natural killer (NK) cells make up in peripheral lymphocytes

A

5 to 10%

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11
Q

What percentage of reactions are nonatopic allergies

A

20-30% of immediate hypersensitivity

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12
Q

What are plasmablasts

A

Antibody secreting cells in the peripheral blood

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13
Q

How does the crosslinking in mast cells lead to compound production

A

1) Cross linking activated phospholipase A2
2) PL A2 take phospholipids into arachidonic acid
3) Activation of prostaglandin and leukotriene pathways

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14
Q

What is the gene that is most commonly associated with autoimmune diseases

A

PTPN22 (because the association with RA)

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15
Q

What are the primary costimulatory molecules for T cells

A

Binding of CD28 on T cells with B7/ CD80/86 on APCS

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16
Q

What cytokines activate NK cells and what are their function

A

IL2, 15 (Stimulate proliferation-NK from T line)

IL-12 (killing and IFN-gamma secretion)

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17
Q

What genes are located in 5q31

A

IL 4, 5, 9, 13, GM-CSF all which have a higher relation to asthma

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18
Q

What is the purpose of proteoglycans (chondroitin sulfate) in mast cells

A

Helps to package and store the amines

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19
Q

Which portion of MHC 2 does CD4 bind to

A

Beta 2

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20
Q

T helper cells use and stimulate other cells via which mechanism

A

Cytokines with CD40L binding to CD40 on APCs

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21
Q

What is occurring during the deposition of immune complexes

A

Antigen-antibody complexes are deposited into tissue. These complexes are of medium size, formed in slight antigen excess, and most pathogenic

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22
Q

What are the characteristics of the TCR

A

Noncovalent linked polypeptides (6 total)

CD3 and zeta chains are invariant (identical) in all cells

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23
Q

Where are C-type Lectin receptors (CTRs) located and what is their ligand

A

Expressed on the plasma membrane of macrophages and dendritic cells, where they detect fungal glycans

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24
Q

Inflamasome activation activates which caspase to form which product

A

Caspase 1 to produce IL-1

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25
Q

What receptors are not expressed on NK cells, and what kind of granules do they have

A

No TCRs or Igs, but contain azurophilic granules

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26
Q

What are the two receptors that help to limit autoreactive T cells

A
  • CTLA4

- PD1

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27
Q

What are the characteristics of the late phase reaction of type 1 hypersensitivity

A

Infiltration of eosinophils, neutrophils, basophils, monocytes and CD4 T cells

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28
Q

In lymph nodes, where are the T cells located

A

Paracortex, next to the follicles

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29
Q

What are the likely sites of antigen-antibody complexes to be deposited

A

Where blood is filtered at high pressure into urine and synovial fluid, aka joints and glomeruli

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30
Q

What are the three phases in Type 3 hypersensitivity

A

1) Formation of immune complexes
2) Deposition of Immune complexes
3) Inflammation and tissue damage

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31
Q

Where are mature T cells found

A

Blood and T cell zones of lymphoid tissue

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32
Q

What are the four circumstances where Ab mediated cell destruction and phagocytosis result in type 2 hypersensitivity

A

1) Transfusion reaction
2) Erythroblastosis fetalis
3) Autoimmune hemolytic anemia
4) Drug reactions

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33
Q

What is a trigger for bronchial asthma

A

Viral infections

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34
Q

What are the characteristics of the MHC 1 chains

A
  • Polymorphic alpha chain

- Nonpolymorphic beta2 chain

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35
Q

If a T cell expresses BIM, what likely happened

A

It was a self reactive T cells that is signaled to undergo apoptosis

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36
Q

What are the domanent cells of chronic inflammation

A

Macrophages

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37
Q

What are the characteristics of the MHC 2 chains

A

Alpha and beta are both polymorphic

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38
Q

For MHC1, which regions make up the peptide binding cleft

A

Alpha 1 and alpha 2

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39
Q

What is the timing fro the immediate reaction in type 1 hypersensitivity

A

Within minutes of allergen exposure

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40
Q

What is that cause of autoimmune lymphoproliferative syndrome (ALPS)

A

Mutations in the FAS receptor gene

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41
Q

What are the 4 major types of innate immune receptors

A

1) TLRs
2) NOD like receptors (NLRs)
3) C-type Lectin receptors (CLRs)
4) Rig-like receptors (RLRs)

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42
Q

What is CD21

A

Complement receptor 2 (CR2)

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43
Q

What is the timing of the late phase reaction in type 1 hypersensitivity

A

2 to 24 hours without the need of antigen exposure

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44
Q

What are the results of the enzymes released by granules in mast cells

A

Tissue damage, Kinin production, activation of complement proteins

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45
Q

What tends to be higher in atopy individuals

A

IgE and Th2 levels

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46
Q

What is the result of vasoactive amines being released

A

1) Smooth Muscle Contraction
2) Increased vascular permeability
3) mucus secretion of nasal, bronchial, and gastric

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47
Q

What is the significance of N-formylmethionyl

A

Initiates the transcription/creation of proteins in bacteria and mitochondria. Allows recognition of bacterial proteins and chemotaxis to them

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48
Q

Where are RIG-like receptors (RLRs) located and what are their ligands

A

Located in the cytoplasm of most cells where they detect viral nucleic acids and prevent replication

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49
Q

What is the condition of erythroblastosis fetalis

A

Antigenic difference between the mother and fetus, where the IgG antierythrocyte antibodies of the mother crosses the placenta and lysis fetal RBCs

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50
Q

What is the function of autoimmune regulator

A

Aka AIRE, which stimulates expression of self antigen

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51
Q

What is the role of prostaglandin D2 in the type 1 immediate hypersensitive reaction

A

Most abundant cyclooxygenase product and produces intense bronchospasms and mucus production

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52
Q

What are the enzymes released by mast cells

A 
Neutral proteases (Chymase, tryptase)
Acid hydrolases
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53
Q

What is responsible for the adhesion of T cells to APCs

A

Integrins

54
Q

What do the HLA for MHC1 code for

A

The polymorphic portion of the Alpha chains

55
Q

What is occuring in the formation of immune complex during type 3 hypersensitivity reactions

A

Protein antigen triggers an immune response, where about a week later, antibodies are made and form the antigen-antibody complex

56
Q

What are systemic autoimmune diseases usually involving and what are the alternative names for it

A

Usually involve blood vessels and connective tissue. This leads to the alternative name of collagen vascular disease or connective tissue disorder

57
Q

What is the function of the alpha3 portion of MHC1

A

It is nonpolymorphic and binds to CD8

58
Q

What is the cause of autoimmune polyendocrinopathy

A

Mutations in the AIRE gene

59
Q

How was peptides processed for MHC1 molecules

A

Cytosolic proteins are taken up and transported into the ER, where they are bound to freshly synthesized MHC

60
Q

What determines being prone to immediate hypersensitivity reactions

A

Genetics

61
Q

What percentage of T cells are CD4+

A

60%

62
Q

What are the three requirements for something to be considered a autoimmune diease

A

1) Presence of immune reaction specific for self antigen or tissue
2) Evidence of damage is the primary pathogenic condition
3) Absence of any other well defined disease

63
Q

What size of stimulus is required to illicit system anaphylaxis

A

Small amounts, where even skin testing amounts can trigger

64
Q

What are the receptors that are characteristic of Natural killer cells (NKs)

A

1) CD16 (Fc receptor for IgG)

2) CD56

65
Q

What type of cells have the antigen receptor genes and which cell lines have rearranged expression

A

All cells have the antigen receptors in the germline, but only immune cells express them, and only B/T lines have rearranged antigen receptors

66
Q

What are the two major products of eosinophils and what are their functions

A

Major basic protein, cationic protein which both damage tissue

67
Q

What is nonatopic allergy

A

Immediate hypersensitivity reactions to non-antigenic stimuli, ugh as extreme temperatures and exercise. This does not involve IgE or Th2 cells

68
Q

Where do plasma cells reside

A

Bone marrow and lymphoid tissue because they do not need to migrate to infection sites

69
Q

A defective PTPN22 results in disease via which mechanism

A

Defective Protein tyrosine phosphatase can not remove phosphate from tyrosines, so tyrosine kinases run unchecked and result in excessive lymphocyte activation

70
Q

What is the primary mechanism responsible for depletion of cells coated with Abs

A

Phagocytosis

71
Q

What are the triggers for activation of mast cells

A

Cross linking of IgE, C5a, C3a (aka anaphylatoxins), IL-8, drugs, and physical stimuli

72
Q

Which lymph node is responsible for the responses to blood borne antigens

A

Spleen

73
Q

What are the cytokines that cause THelpers to differentiate into Th17

A

IL1, 7, 23(close relative of 12)

74
Q

What is the treatment for late phase type 1 hypersensitivity

A

Broad spectrum anti inflammatory drugs (steroids) rather than antihistamine (the mediator of the first phase)

75
Q

What are the 3 stages involved in the innate immune system

A

1) Recognition of the microbe/damage
2) Activation
3) Elimination of unwanted substances

76
Q

What is the function of dendritic cells

A

In the Initiation of the immune response via presentation, but not in the destruction of pathogens

77
Q

What is the most important mast cell derived amine

A

Histamine

78
Q

What infection is highly associated with atopic dermatitis

A

Bacterial skin infection

79
Q

What percent of lymphocytes in the blood does mature T cells make up

A

60-70%

80
Q

Which disease is associated with HLA-A

A

Hereditary hemochromatosis

81
Q

How is antigen receptor diversity created

A

Somatic recombination of genes that code for receptor proteins

82
Q

What is the main location of isotype switching

A

Germinal centers

83
Q

What is the treatment for autoinflammatory syndromes

A

IL-1 antagonists

84
Q

What is the function of recombination activating genes (RAG 1 and 2) and what is the result of a defect

A

Responsible for the recombination of gene segments in the creation of lymphocyte receptors. Defects result in lack of mature lymphcytes

85
Q

What is the conditions of autoimmune hemolytic anemia, granulocytosis and thrombocytopenia based from

A

Autoantibodies against their own blood/platelet cells

86
Q

Polymorphism with PTPN22 are associated with which disorders

A

RA, Type 1 diabetes, other autoimmune

87
Q

What is the cause and result of autoinflammatory syndromes

A

Gain of function mutations in the NLRs result in periodic fever syndromes

88
Q

What is the postulated mechanism for uveitis and post-traumatic orchitis

A

Damage or traumatic causing the release of sequestered antigens

89
Q

What does contact with urushiol

A

The antigenic component of poison oak, causes vascular dermatitis

90
Q

What is the result in FOXP3

A

Autoimmune disorders, leading to a condition known as Immune dysregulation, polyendocrinopathy, enteropathy, X linked, or IPEX

91
Q

What are the two transcription factors that are activated by TLRs

A

1) NFkB (Cytokine synthesis and adhesion molecules for leukocyte recruitment)
2) interferon regulatory factors (IRFs)- production of type on interferons (alpha and beta)

92
Q

Polymorphism in the gene NOD2 results in which disease

A

Crown disease

93
Q

Where are eosinophils derived from

A

Bone marrow

94
Q

What are the characteristic of systemic anaphylaxis

A

Vascular shock, widespread edema, difficulty breathing

95
Q

What percentage of T cells are alpha Beta TCR

A

95% of T cells

96
Q

What is perivascular cuffing

A

Accumulation of CD4 T cells and macrophages in the perivasculature

97
Q

What are the two mechanisms of Type 4 hypersensitivity

A

1) CD4 mediated inflammation (major component)

2) CD8 destruction

98
Q

What is the family of activating receptors on NK cells and what do they respond to

A

NKG2D, and respond to infection and DNA damage

99
Q

Necrotizing vasculitis aka fibrinoid necrosis are characteristic of which type of hypersensitivity

A

Type 3 hypersensitivity

100
Q

In the spleen, where are the T cells located

A

Periarteriolar lymphoid sheath

101
Q

What is the cause of allergic asthma

A

Late phase type 1 reaction

102
Q

What is the relationship between HLA-B27 and ankylosing spondylitis

A

100-200 times more likely to develop disease with this HLA

103
Q

What mechanism of cytokines normally work through

A

Autocrine and paracrine, and very rarely endocrine

104
Q

During a type 4 hypersensitivity, what is the function of TH1 and Th17

A 
Th1= activation of macrophages
Th17= Activation of neutrophils
105
Q

What is the immediate reaction of a type 1 reaction characterized by

A

Vasodilation, vascular leakage, smooth muscle spasm or glandular secretion

106
Q

What are the results of late phase type 1 hypersensitivity reactions

A

-Bronchospasms, edema, Mucus secretion, smooth muscle spasms

107
Q

What is the major lipid mediators in immediate hypersensitivity

A

Arachidonic acid

108
Q

Where are cutaneous and mucosal lymphoid systems located

A

Epithelia of skin,GI and respiratory systems

109
Q

What receptor does EBV use to enter and infect B cells

A

Complement receptor 2

110
Q

What are the results seen from inflammation in type 3 hypersensitivity

A

Fever, hives (urticaria), joint pain (arthralgias) LN enlargement, proteinuria

111
Q

What percentage of circulating peripheral lymphocytes do mature B cells make up

A

10-20%

112
Q

During inoculation of PPD, what is the accumulation of cells characterized by

A

PPD aka, tuberculin causes Mononulcear cells (CD4 cells, and macrophages)

113
Q

What are the locations of TLRs

A

Plasma membrane and endosomal vesicles

114
Q

Atopy individuals have what percentage of family history of allergies

A

50%

115
Q

What are the three locations of PRRs

A

Cytosol, endoscope, membranes

116
Q

What are the phagocytes in the blood

A

Monocytes and neutrophils

117
Q

What are the three things released from the granules of activated mast cells

A

Vasoactive amines, enzymes, proteoglycans

118
Q

What potion of the MHC2 make up the peptide binding cleft

A

Alpha 1 and beta 1

119
Q

What are the location of basophils

A

In blood circulating in small amounts

120
Q

How does polymorphism in the NOD2 gene result in inflammatory bowel syndromes

A

Mutation in MOD2, so it is unable to detect commensal bacteria, so there is chronic inflammation

121
Q

How does EBV and HIV lead to autoimmunity

A

Causes polyclonal activation and autoantibodies by releasing self antigens, then structurally altering them so they are able to activate T cells

122
Q

What is atopy

A

Increased propensity to develope immediate hypersensitivity reactions

123
Q

What does the gene PTPN22 code for

A

Protein tyrosine phosphatase

124
Q

Which disease is associated with HLA-BW47

A

21-hydroxylase deficiency

125
Q

What are the ligands for the NOD-like receptors (NLRs)

A

1) Necrotic cell byproducts (ATP,uric acid)
2) Ion Disturbances (potassium loss)
3) Microbial products

126
Q

What is fibrinoid necrosis associated with

A

Immune complex injury

127
Q

What is epitope spreading

A

Immune responses to one self antigen causes the release of other antigens and newly activated self lymphocytes

128
Q

What is the role of surfactant in immune protection

A

In the lungs and serves as an innate component against inhaled microbes

129
Q

In the spleen, where are the B cells located

A

Follicles

130
Q

Many NLRs activate inflammation via which structure

A

Inflammasomes

Robbins Pathology MOD1 (Chptr 1-6) (10 decks)

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