FINAL HY Flashcards

1
Q

Which cytokine is responsible for weakening tight junctions and Hemopexin (sequesters iron)

A

IL-22

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2
Q

Pre-BCR signaling activates ____ which promotes survivial and proliferation

A

Btk

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3
Q

X-linked Agammaglobulinemia is due to a mutant version of ___ which results in lack of mature B cell production and antibodies
–> High Risk of infections – need Ig therapy for life

A

Btk

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4
Q

Which B cells migrate into the follicle to interact with follicular DC (FDC) to become mature B Cells?

A

T2 Transcriptional B Cells

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5
Q

If the H chain has recombined properly, the developing B cell passes the checkpoint and becomes what cell?

A

Large Pre B Cell

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6
Q

Cluster of BCR indicates what?

A

BCR Crosslinking occurs

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7
Q

What are the signaling molecules of the BCR that contain cytoplasmic tails with ITAMs?

A

Ig-Alpha and Ig-Beta

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8
Q

What binds to P-ITAMS and acts similar to ZAP-70 in T cells?

A

Syk (Spleen Tyrosine Kinase)

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9
Q

This B-cell coreceptor recognizes iC3b and C3d derivatives of C3b fragments…

Keeps it Together

A

CR2 (CD21)

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10
Q

This B-cell co receptor is the singaling chain of the co-receptor…

A

CD-19

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11
Q

This B-cell co receptor binds CD19 and aggregates the co-receptor and BCR…

A

CD81

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12
Q

Patients who have low levels of serum antibodies, limited isotype switching and poor responses to infections and vaccinations are lacking what B-cell co receptors?

A

CD19 and CD81

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13
Q

Follicular Zone B cells require interaction with which T cells?

A

CD4+ T Cells

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14
Q

A 1-year old boy with a history of severe infections is found to have very few circulating mature T cells or NK cells but normal numbers of B cells. Genetic studies reveal he has X-linked severe combined immunodeficiency syndrome. Defective signaling by the receptor for which cytokine is the underlying cause of this disease?

A

only IL-7 is required for early development of T cells and NK cells in the thymus of humans

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15
Q

B cells engage Ag contentrated by ___ and are activated

A

FDC

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16
Q

Pre-TFH cells are activated by Ag presented by what?

A

DC

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17
Q

Pre-TFH cells can produce survival cytokines and encourage proliferation through what

A

Activation Induced Deaminase
AID

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18
Q

B cells promote differentaiton of Pre-TFH cells into TFH cells via induction of what?

A

BCL-6

induces production of IL-10 and IL21, 21R

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19
Q

Rapidly dividng B cells in germinal center =

A

Centroblasts

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20
Q

What promotes somatic hypermutations in centroblasts, which results in Igs with varying affinities for Ag

A

AID

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21
Q

Centroblasts that have completed somatic hypermutation and now express a bCR (slg) =

A

Centrocytes

engage with FDC – Affinty Maturation

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22
Q

What cytokines promote proliferation and differentation of GC B cells into plasma cells?

A

IL-10 and IL21 (signaling Bcl-6)

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23
Q

What cytokine promotes plasma cell differenation?

A

IL10

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24
Q

What cytokine promotes memory B cell differentation?

A

IL4

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25
Q

proteins bound to mucin which proovides a physical barrier that is difficult for pathogens to pass through

A

Glycocalycx

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26
Q

What antimicrobial peptides are made by paneth cells in the SI?

A

Alpha Defensins
Beta defensions are in the LI

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27
Q

these C-type lectins are made by paneth cells which block bacterial colonization and also have gram + bactericial effects

A

REG III
regenerating islet-derived proteins

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28
Q

epithelial cells produce these in response to injury or pathogenic microbes

A

ALARMINS
Innate Lymphoid cells respond to these by secreting cytokines

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29
Q

What expression is reduced to prevent responses to commensal bacteria

A

TLR4 expression

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30
Q

The salivary glands which are the main source of IgA in the oral cavity are part of which MALT?

A

D-MALT

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31
Q

When a mucosal epithelial cell becomes infected, injured or stressed is expresses what proteins so that gamma-delta cells bind and kill that cell?

A

MICA/MICB

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32
Q

Which is an Inductive Site and Effector Site?

Peyers Patch Vs. Lamina Propria

A

Peyers Patch = Inductive
Lamina Propria = Effector (secretion of ab via plgR)

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33
Q

Where does clonal proliferation and differentiation to memory mlgA B cells occur?

A

Messenteric Lymph Node

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34
Q

this ADAPTIVE mechanism is seen in response to what pathogen

MHC II → production of IL-12 differentiates CD4+ into TH1 cells to produce cytokines IFN-gamma/IL2

A

Intravesicular Bacteria = stuck in phagolysosome

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35
Q

Innate – Extracellular Fungi

What are the most important cells of a fungal infection to kill in their phagolysosome?

A

Neutrophils

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36
Q

Adaptive Response to Extracellular Fungi

What recptor on DC detects fungal glucans, and then produces what cytokines?

A

Dectin-1
Cytokines: Il-1, IL-6, IL-23 which promote CD4+ T cell differenation into TH17

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37
Q

Innate Response to Viruses

Virus infected cells produce what interferons which induces an antiviral state?

A

Type 1 INF alpha and Beta

Inc MHC1 expression
Activate NK

Induce resistance to viral replication

38
Q

these vaccines are live, attenuated vaccines with lots of potential to prevent respiratory diseases since the airway has lots of IgA
Promotes a Mucosal Response

A

Nasal Vaccines

39
Q

Which vaccine develops HUMORAL and CMI responses?

A

Live Attenuated Vaccines

40
Q

Which vaccine develops only the humoral response?

A

Inactivated Vaccine

41
Q

What type of hypersensitivy reactions are immediate

A

TYPE 1 – Allergies

42
Q

In Type 1 hypersensitivity reaction, upon second exposure the allergen binds to the** IgE –> multiple FcER** engagments crosslink initiating what ?

A

Mast Cell Activation

43
Q

What type of hypsersensitivty reaction is antibody mediated?

A

Type 2 Hypersensitivity
Cytotoxic

44
Q

What is it called when cells cannot engulf tissues expressing ag and what happens

A

Frustrated Phagocytosis
neutrophils expell/MAC attack

45
Q

AntiGBM disease
General, lung and renal symptoms
IgG against NC1 domain of type IV collagen (a3 chain)

A

Good Pastures Syndrome

46
Q

In Hemolytic Disease of Newborn what do you expect to see regarding Hb and billirubin levels?

A

DEC Hb and INC in billirubin

47
Q

Name the Disease

Driven by TH2 response (IL4 production)
Agonistic anti-TSHR IgG binds to TSHR site → HYPERthyroidism

A

Graves Disease

48
Q

Name the Disease

IgG blocks cell functioning associated with protein target
Binds to Ach R/prevents binding
Drooping Eyes, facial/limb weakness

Antagonisitic

A

Myasthenia Gravis

49
Q

What hypersensitivity reaction is immune complex mediated

Response to soluble Ag and forms IC

A

Type III

50
Q

Patients deficient in Factor I, C1, or C3 are susceptible to what reaction

This would cause reduce in IC clearance

A

Hypersensitivity TYPE 3

51
Q

Ab Mediated IC – human ab response against the horse ab leading to IC formation

A

Serum Sickness

52
Q

localized IC formation and vasculitis associated with a repeated Ag exposure (vaccine boosters)

Symptoms driven by C3a and C5a

A

Arthus Reaction

53
Q

multiple ab directed against Fc region of IgG – Inflammatory

A

Rheumatoid Factor

54
Q

Systemic Lupus Erythematosus

Normal Apoptotic body clearance is done via

A

C1q, MBL, and pentraxins to avoid inflammation

Deficiencies in these would lead to NECROSIS

55
Q

Which hypsensitivty reaction is T-Cell Mediated/Delayed

A

Type 4 Hypersensitivity

56
Q

In contact hypersensitivity, during the sensitization phase conjugates are formed between allergen (hapten) and skin protein –> what cells are generetaed and traffic to the skin tissue

A

Effector Memory TH1 cells

57
Q

What is the second response in contact hypersensitivity?

A

Effector Phase = Macrophage Activity

58
Q

What cells secrete large amounts of fibrolast activating factor to stimulate fibroblasts to produce a fibrous capsule to quarantine the microbe

A

Epitheliod Cells

59
Q

Lipid bodies accumulate due to microbial stimulation of foamy cell TLR and action of TNF-alpha and MCP-1
Maintain microbe in a non-replicating state

A

Foamy Cells

60
Q

Cellular Primary Immunodeficiency

caused by a mutation in genes for perforin-granzyme killing by CTLs and NK cells —> Overactivation of Macrophages

Excessive inflammation/Phagocytosis of RBC

A

Hemphagocytic Lymphohistiocytosis (HLH)

61
Q

Humoral Primary Immunodeficiency = MOST COMMON

defect in B cell developement –> IgA due to differentation defect

Recurrent mucosal infections

A

Selective IgA deficiency

Cant deliver IgA d/t no delivery mechanism

62
Q

Humoral Primary Immunodeficency

no B cell differentation, so number of B cells declines over time , lack of memory cells

Risk of pyogenic bacterial infection

A

Common Variable Immunodeficiency

63
Q

Humoral Primary Immunodeficency

mutation in AID, lack of switched isotypes, ratios are off

hemolytic anemia

A

Activation induced deaminase hyper IgM syndrome 2

64
Q

SCID

patients fail to develop thymus
Lack of T Cells
Lower # of memoral pheriphery B Cells

Low Set Ears, Fish Mouth, Notched Ear pinnae

A

DiGeorge Syndrome

65
Q

SCID

lack of MHC1/2 due to a mutation in Tap 1/2 or mutation in transcription factors
Lack of circulating CD4 or CD8 alpha beta thymocytes

1 – resp bac. infc.
2 – bac,viral or fungal infc.

A

Bare Lymphocyte Syndrome

66
Q

Phagocytic Primary Immunodeficency

dysfunction of killing enzyme in phagolysosome

Phagocytic cells fuse and form granulomas

A

Chronic Granulomatous Disease

67
Q

Which Leukocyte Adhesion Deficiency?

mutation in CD18 gene
integrin needed for extravasion

poor wound healing usually before 1yo

A

LAD 1

68
Q

Which Leukocyte Adhesion Deficiency?

mutation in sialyl-lewis X in neutrophils

Low PMN migration

A

LAD 2

69
Q

Which Leukocyte Adhesion Deficiency?

mutation in signaling pathway for upregulation of selectins on leukocytes

defective platelet aggregation – bleeding disorders

A

LAD 3

70
Q

Leukemias vs Lymphomas

A

leukemias = proliferate as indv. cells
Lymphomas = proliferate as a mass of cells

71
Q

What type of leukemia?

dysregulated proliferation/clonal expansion of lymphoid progenitor

MC malignancy in children

A

ALL = Acute Lymphocyte Leukemia

72
Q

What type of leukemia?

B Cells cannot undergo apoptosis d/t genetic alteration

Adults 55+

A

CLL - Chronic Lymphocytic Leukemia

73
Q

What type of leukemia?

malignant proliferation of immature granulocyte precursor cells

leads to lack of RBC/platelets

A

Acute Myeloid Leukemia

74
Q

What type of leukemia?

malignant plasma cells that accumulate in bone – bone is destroyed – punched out lytic lesions

BENCE Jones Proteins (+ prod of L Chains passing through kidneys

A

Multiple Myeloma

75
Q

What type of leukemia?

Neoplastic hyper IgM syndrome
Malignant plasma cells produce IgM – serum thickens and impairs blood

A

Waldenstrom macroglobulinemia

76
Q

What type of lymphoma?

Spread from LN to LN in orderly/predicatable fashion
Often begins near neck

Reed Sternberg Cells Teens/YA

A

Hodgkins Lymphoma

77
Q

T Cells require two signals for activation

A

TCR with APC + CD28 to B7

78
Q

Negative Selection Outcome

strong binding of self ag with BCR

A

APOPTOSIS
d/t persisten RAG1/2 expression –> rearrangement of L chain DNA

79
Q

Negative Selection Outcome

strong binding of soluble self-ag without BCR cross linking –>

A

ANERGY
cells survive and enter the periphery but are unresponsive

80
Q

During single positive selection, single positive T cells engage with what?

A

MTEC = medullary thymic epithelial cells
MTEC produces all self antigens by expression of AIRE gene

81
Q

transcription factor that induces expression of suppressor molecules

A

Foxp3

82
Q

constitutive expression of _______
required for Treg cell development, if deficent or Foxp3 deficient, then systemic autoimmunity develops

A

CD 25

83
Q

What type of rejection

Occurs within 24 hours and is mediated by circulating antibodies

incorrect ABO typing

A

Hyperacute Rejection

84
Q

Type of Rejction?

Occurs within 1-3 weeks and is mediated by B + T cells targeting endothelial tissue
main cause is immunosupressive drugs given before/after transplant

A

Acute Rejection

85
Q

Type of Rejection

within months to yaers, mediated by immune complexes and T cells

A

Chronic Rejection

86
Q

What Autoimmune disease?

immune complex deposition = chronic T3HSR
Most patients expect flares caused by different triggers
Raynauds

A

Systemic Lupus Erthematousus

87
Q

What autoimmune disease

Mediated by T cell attack on myelin
Vision Issues = Most Common initial symptom

A

Multiple Sclerosis

88
Q

What autoimmune disease

Antagonistic auto-abs against AchR on motor endplates of muscles blocking signal to muscle
Ptosis and diplopia

A

Myasthenia Gravis

89
Q

What autoimmune disease>?

Auto-abs against thyroid proteins
Weight Gain, cold sensitivity

A

Hashimotos Thyroiditis

90
Q

What autoimmune disease

Agonistic auto abs to TSH receptor – cont. stimulation = hyperthyroidism
Hand tremor, heat sensitivity, weight loss

A

Graves Disease

91
Q

Treatment for Graves dermopathy

A

Beta Blockers

92
Q

CTLs infiltrate islets of langerhans and damage insulin producing cells by releasing cytokines and lytic enzymes

A

Type 1 insulin dependent diabetess