FINAL HY

Question 1

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

Answer 1

IL-22

Question 2

Pre-BCR signaling activates ____ which promotes survivial and proliferation

Answer 2

Btk

Question 3

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

Answer 3

Btk

Question 4

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

Answer 4

T2 Transcriptional B Cells

Question 5

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

Answer 5

Large Pre B Cell

Question 6

Cluster of BCR indicates what?

Answer 6

BCR Crosslinking occurs

Question 7

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

Answer 7

Ig-Alpha and Ig-Beta

Question 8

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

Answer 8

Syk (Spleen Tyrosine Kinase)

Question 9

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

Keeps it Together

Answer 9

CR2 (CD21)

Question 10

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

Answer 10

CD-19

Question 11

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

Answer 11

CD81

Question 12

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?

Answer 12

CD19 and CD81

Question 13

Follicular Zone B cells require interaction with which T cells?

Answer 13

CD4+ T Cells

Question 14

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?

Answer 14

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

Question 15

B cells engage Ag contentrated by ___ and are activated

Answer 15

FDC

Question 16

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

Answer 16

DC

Question 17

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

Answer 17

Activation Induced Deaminase
AID

Question 18

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

Answer 18

BCL-6

induces production of IL-10 and IL21, 21R

Question 19

Rapidly dividng B cells in germinal center =

Answer 19

Centroblasts

Question 20

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

Answer 20

AID

Question 21

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

Answer 21

Centrocytes

engage with FDC – Affinty Maturation

Question 22

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

Answer 22

IL-10 and IL21 (signaling Bcl-6)

Question 23

What cytokine promotes plasma cell differenation?

Answer 23

IL10

Question 24

What cytokine promotes memory B cell differentation?

Answer 24

IL4

Question 25

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

Answer 25

Glycocalycx

Question 26

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

Answer 26

Alpha Defensins Beta defensions are in the LI

Question 27

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

Answer 27

REG III regenerating islet-derived proteins

Question 28

epithelial cells produce these in response to injury or pathogenic microbes

Answer 28

ALARMINS Innate Lymphoid cells respond to these by secreting cytokines

Question 29

What expression is reduced to **prevent responses to commensal bacteria**

Answer 29

TLR4 expression

Question 30

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

Answer 30

D-MALT

Question 31

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

Answer 31

MICA/MICB

Question 32

# Which is an Inductive Site and Effector Site? Peyers Patch Vs. Lamina Propria

Answer 32

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

Question 33

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

Answer 33

Messenteric Lymph Node

Question 34

# 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

Answer 34

Intravesicular Bacteria = stuck in phagolysosome

Question 35

# Innate -- Extracellular Fungi What are the most important cells of a fungal infection to kill in their phagolysosome?

Answer 35

Neutrophils

Question 36

# Adaptive Response to Extracellular Fungi What recptor on DC detects fungal glucans, and then produces what cytokines?

Answer 36

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

Question 37

# Innate Response to Viruses Virus infected cells produce what interferons which **induces an antiviral state**?

Answer 37

Type 1 INF alpha and Beta Inc MHC1 expression Activate NK | Induce resistance to viral replication

Question 38

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

Answer 38

Nasal Vaccines

Question 39

Which vaccine develops HUMORAL and CMI responses?

Answer 39

Live Attenuated Vaccines

Question 40

Which vaccine develops only the humoral response?

Answer 40

Inactivated Vaccine

Question 41

What type of hypersensitivy reactions are immediate

Answer 41

TYPE 1 -- Allergies

Question 42

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

Answer 42

Mast Cell Activation

Question 43

What type of hypsersensitivty reaction is antibody mediated?

Answer 43

Type 2 Hypersensitivity Cytotoxic

Question 44

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

Answer 44

**Frustrated Phagocytosis** neutrophils expell/MAC attack

Question 45

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

Answer 45

Good Pastures Syndrome

Question 46

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

Answer 46

DEC Hb and INC in billirubin

Question 47

# Name the Disease Driven by TH2 response (IL4 production) **Agonistic** anti-TSHR IgG binds to TSHR site → **HYPERthyroidism**

Answer 47

Graves Disease

Question 48

# Name the Disease **IgG blocks** cell functioning associated with protein target Binds to **Ach R/prevents binding** Drooping Eyes, facial/limb weakness | Antagonisitic

Answer 48

Myasthenia Gravis

Question 49

What hypersensitivity reaction is immune complex mediated | Response to soluble Ag and forms IC

Answer 49

Type III

Question 50

Patients deficient in Factor I, C1, or C3 are susceptible to what reaction | This would cause reduce in IC clearance

Answer 50

Hypersensitivity TYPE 3

Question 51

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

Answer 51

Serum Sickness

Question 52

localized IC formation and vasculitis associated with a repeated Ag exposure (vaccine boosters) Symptoms driven by C3a and C5a

Answer 52

Arthus Reaction

Question 53

multiple ab directed against Fc region of IgG – Inflammatory

Answer 53

Rheumatoid Factor

Question 54

# Systemic Lupus Erythematosus Normal Apoptotic body clearance is done via

Answer 54

C1q, MBL, and pentraxins to avoid inflammation | Deficiencies in these would lead to NECROSIS

Question 55

Which hypsensitivty reaction is T-Cell Mediated/Delayed

Answer 55

Type 4 Hypersensitivity

Question 56

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

Answer 56

Effector Memory TH1 cells

Question 57

What is the second response in contact hypersensitivity?

Answer 57

Effector Phase = Macrophage Activity

Question 58

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

Answer 58

Epitheliod Cells

Question 59

**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**

Answer 59

Foamy Cells

Question 60

# 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

Answer 60

Hemphagocytic Lymphohistiocytosis (HLH)

Question 61

# Humoral Primary Immunodeficiency = MOST COMMON defect in B cell developement --> IgA due to differentation defect | Recurrent mucosal infections

Answer 61

Selective IgA deficiency | Cant deliver IgA d/t no delivery mechanism

Question 62

# Humoral Primary Immunodeficency no B cell differentation, so number of B cells declines over time , lack of memory cells | Risk of pyogenic bacterial infection

Answer 62

Common Variable Immunodeficiency

Question 63

# Humoral Primary Immunodeficency mutation in AID, lack of switched isotypes, ratios are off | hemolytic anemia

Answer 63

Activation induced deaminase hyper IgM syndrome 2

Question 64

# SCID patients fail to develop thymus Lack of T Cells Lower # of memoral pheriphery B Cells | Low Set Ears, Fish Mouth, Notched Ear pinnae

Answer 64

DiGeorge Syndrome

Question 65

# 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.

Answer 65

Bare Lymphocyte Syndrome

Question 66

# Phagocytic Primary Immunodeficency dysfunction of killing enzyme in phagolysosome | Phagocytic cells fuse and form granulomas

Answer 66

Chronic Granulomatous Disease

Question 67

# Which Leukocyte Adhesion Deficiency? mutation in CD18 gene integrin needed for extravasion | poor wound healing usually before 1yo

Answer 67

LAD 1

Question 68

# Which Leukocyte Adhesion Deficiency? mutation in sialyl-lewis X in neutrophils | Low PMN migration

Answer 68

LAD 2

Question 69

# Which Leukocyte Adhesion Deficiency? mutation in signaling pathway for upregulation of selectins on leukocytes | defective platelet aggregation -- bleeding disorders

Answer 69

LAD 3

Question 70

Leukemias vs Lymphomas

Answer 70

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

Question 71

# What type of leukemia? dysregulated proliferation/clonal expansion of lymphoid progenitor | **MC malignancy in children**

Answer 71

ALL = Acute Lymphocyte Leukemia

Question 72

# What type of leukemia? B Cells cannot undergo apoptosis d/t genetic alteration | **Adults 55+**

Answer 72

CLL - Chronic Lymphocytic Leukemia

Question 73

# What type of leukemia? malignant proliferation of immature granulocyte precursor cells | leads to lack of RBC/platelets

Answer 73

Acute Myeloid Leukemia

Question 74

# 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**

Answer 74

Multiple Myeloma

Question 75

# What type of leukemia? Neoplastic hyper IgM syndrome Malignant plasma cells produce IgM -- serum thickens and impairs blood

Answer 75

Waldenstrom macroglobulinemia

Question 76

# What type of lymphoma? Spread from LN to LN in orderly/predicatable fashion Often begins near neck | **Reed Sternberg Cells** Teens/YA

Answer 76

Hodgkins Lymphoma

Question 77

T Cells require two signals for activation

Answer 77

TCR with APC + CD28 to B7

Question 78

# Negative Selection Outcome strong binding of self ag with BCR

Answer 78

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

Question 79

# Negative Selection Outcome strong binding of soluble self-ag without BCR cross linking -->

Answer 79

ANERGY cells survive and enter the periphery but are unresponsive

Question 80

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

Answer 80

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

Question 81

transcription factor that induces expression of suppressor molecules

Answer 81

Foxp3

Question 82

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

Answer 82

CD 25

Question 83

# What type of rejection Occurs within 24 hours and is mediated by circulating antibodies | incorrect ABO typing

Answer 83

Hyperacute Rejection

Question 84

# 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

Answer 84

Acute Rejection

Question 85

# Type of Rejection within months to yaers, **mediated** by **immune complexes and T cells**

Answer 85

Chronic Rejection

Question 86

# What Autoimmune disease? immune complex deposition = chronic T3HSR Most patients expect flares caused by different triggers Raynauds

Answer 86

Systemic Lupus Erthematousus

Question 87

# What autoimmune disease Mediated by T cell attack on myelin Vision Issues = Most Common initial symptom

Answer 87

Multiple Sclerosis

Question 88

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

Answer 88

Myasthenia Gravis

Question 89

# What autoimmune disease>? Auto-abs against thyroid proteins Weight Gain, cold sensitivity

Answer 89

Hashimotos Thyroiditis

Question 90

# What autoimmune disease Agonistic auto abs to TSH receptor -- cont. stimulation = hyperthyroidism Hand tremor, heat sensitivity, weight loss

Answer 90

Graves Disease

Question 91

Treatment for Graves dermopathy

Answer 91

Beta Blockers

Question 92

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

Answer 92

Type 1 insulin dependent diabetess