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MOD Exam 2 > Diseases of the Immune System > Flashcards

Diseases of the Immune System Flashcards

1
Q

What is the principal morphologic manifestation of immune complex injury (i.e. injury from type III hypersensitivity rxn)?

A

Acute vasculitis with associated necrosis.

Necrotic tissue appears fibrinoid in histologic slides (fibrinoid necrosis)

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2
Q

Isotype switching is induced by what cytokines?

A

IFN-Υ

IL-4

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3
Q

Immune-complex mediated diseases tend to be systemic, but often prefernetially involve what areas of the body?

A

Kidneys

Joints

Small blood vessels

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4
Q

In the spleen, where are B cells and T cells located?

A

B cells located within follicles

T cells are concentrated in periarteriolar sheaths surrounding small arterioles

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5
Q

Where are TLRs located and what are their functions?

A

In the plasma membrane and endosomal vesicles

Recognize different sets of microbes

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6
Q

What is the clinical presentation of a patient who has progressed to the development of AIDS

A

Fever

Fatigue

Weight loss

Diarrhea

Opportunistic infections

Secondary neoplasms

Neurological deficits

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7
Q

Function of type I interferons in the antiviral defense initiated by innate immunity

A

Act on infected and uninfected cells to activate enzymes that degrade viral nucleic acids and inhibit viral replication, inducing the antiviral state

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8
Q

What accounts for the majority of deaths in untreated AIDS patients?

A

Opportunistic infections

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9
Q

What type of mutation in TLRs is associated with rare but serious immunodeficiency syndromes?

A

Germline loss-of-function mutation

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10
Q

Clinical manifestations of DiGeorge syndrome

A

Facial abnl

Cleft palate

Tetany 2/2 hypocalcemia

Immune deficiency 2/2 T cell deficiency as a result of thymic hypoplasia

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11
Q

Type I hypersensitivity rxns have two phases, what are they?

A

Immediate reaction

Late-phase reaction

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12
Q

infants affected by SCID present with

A

thrush, diaper rash and failure to thrive

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13
Q

How does UV irradiation exacerbate SLE in certain individuals?

A

UV light may induce apoptosis in cells

May stimulate keratinocytes to produce IL-1

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14
Q

How do the two types of dendritic cells help to maintain the HIV infection?

A

Mucosal dendritic cells are used to transport infection to lymph nodes

In the lymph nodes, follicular dendritic cells act as additional reservoirs of infection

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15
Q

Drug-incuced lupus has what positive ab test?

A

ANA

Anti-histone Ab

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16
Q

How is it possible to detect polyclonal (nonneoplastic) lymphocyte proliferation from monoclonal (neoplastic) lymphoid tumors?

A

Each T cell or B cell and its clonal progeny have a unique DNA arrangment and a unique Ag receptor which can be detected by molecular analysis

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17
Q

What type of cells are effectively destroyed via the MAC, generated through the activation of the complement system?

A

Cells with thin walls, such as Neisseria bacteria

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18
Q

What causes most type I hypersensitivity rxns?

A

Excessive Th2 cell responses

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19
Q

Although macrophages allow for viral replication of HIV, they are resistant to the cytopathic effects of the virus. Instead, macrophages act as a ___ of infection

A

Reservoir

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20
Q

RIG-like receptor location and function

A

Cytosol of most cell types

Detect nucleic acids of viruses and replicate in the cytoplasm of infected cells

Stimulate production of antiviral cytokines

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21
Q

Clinical manifestations of chediak-higashi syndrome

A

Increased susceptibility to infection

Neutropenia

Giant granules within leukocytes

Albinism 2/2 defects in melanocytes

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22
Q

What gene is responsible for initiating transcription and binds TFs?

A

LTR (long terminal repeat)

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23
Q

C-type lectin receptor location and function

A

Plasma membrane of macrophages and DCs
Detect fungal glycans and elicit inflammatory rxns to fungi

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24
Q

What is the most common form of parenteral transmission of HIV?

A

Intravenous drugs, through sharing of needles

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25
Q

What are the major components of innate immunity

A

Epithelial barriers

Phagocytic cells (neutrophils, MΦ)

Dendritic cells

NK cells

Plasma proteins, including complement

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26
Q

What leukocyte is seen in abundant amounts during a late-phase rxn?

A

Eosinophils

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27
Q

Function of CD4+ T-cells

A

secrete cytokines that assist macrophages and B lymphocytes in combatting infection

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28
Q

Acute cellular rejection, often seen within the initial months post-transplantation, is mediated by what cell type?

A

CD4+ T-cells, generating cytokines that promote an inflammatory response and graft injury via activated macrophages

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29
Q

What are the subtypes of CD4+ T-cells

A

TH1

TH2

TH17

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30
Q

What function do integrins have in T-cell activation?

A

Promote the attachment of T-cells of APCs

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31
Q

Arthus reaction

A

localized area of tissue necrosis resulting from acute immune complex vasculitis, usually elicited by the skin

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32
Q

Where are dendritic cells located and what is their function?

A

Epithelia, lymphoid organs, most tissues

Capture antigens and display peptides for recognition by T-cells

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33
Q

SLE predominantly affects what gender?

A

Females

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34
Q

What is characteristic of the limited scleroderma, CREST syndrome?

A

Calcinosis (in skin)

Raynaud’s

Esophageal dysfunction (GERD, decreased motility)

Sclerodactyly

Telangiectasis (dilated capillaries)

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35
Q

What are the classic primary innate immunodeficiency disorders associated with leukocytes?

A

Chediak Higashi syndrome

Chronic granulomatous disease

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36
Q

What occurs in central tolerance?

A

Immature self-reactive B and T cell clones that recognize self-Ags during their maturation in primary lymphoid organs are killed or rendered harmless

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37
Q

Function of IL-1

A

Mediator of inflammation, recruiting leukocytes to site of infection

Induction of fever

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38
Q

A patient’s serum will show what is they have Hyper-IgM syndrome?

A

High levels of IgM

No IgA or IgE

Extremely low levels of IgG

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39
Q

What cytokine is involved in the antiviral defense

A

Type I Interferons

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40
Q

Ab-mediate inflammation is the mechanism responsible for tissue injury in what types of disorders?

A

Glomerulonephritis

Vascular rejection in organ grafts

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41
Q

Affinity maturation

A

When helper T-cells stimulate the production of antibodies with high affinity for Ag

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42
Q

How does a T-cell become anergic?

A

When an Ag is presented to a T-cell but there is inadequate level of co-stimulation

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43
Q

How can an amyloid be differentiated from other hyaline materials?

A

Through the use of Congo red stain

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44
Q

Polymorphisms in what gene are associated with rheumatoid arthritis, type 1 DM and other diseases?

A

PTPN22

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45
Q

Stages of the innate immune response

A

recognition of microbes and damaged cells

activation of various mechanisms

elimination of unwanted substance

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46
Q

What is the hallmark of AIDS?

A

profound immune deficiency, specifically a marked reduction of CD4+ T-cells

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47
Q

What is the life cycle of HIV?

A

Infection

Integration of provirus into host cell genome

Activation of viral replication

Production and release of infectious virus

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48
Q

What tumors are of high incidence in patients with AIDS?

A

Kaposi sarcoma

B-cell lymphoma

Cervical CA in women

Anal CA in men

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49
Q

Explain the process of clonal selection of lymphocytes

A

Lymphocytes specific for a large number of Ags exist before exposure to Ag, and when an Ag enters, it selectively activates the Ag-specific cells

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50
Q

What types of preformed mediators are released during an immediate hypersensitivity rxn?

A

Vasoactive amines (histamine)

Enzymes, including neutral proteases and acid hydrolases

Proteoglycans including heparin and chondroitin sulfate

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51
Q

The innate immune system provides host defence through two main reactions, what are they?

A

Inflammation

Antiviral defense

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52
Q

CD8 T cells bind what class of MHC molecules?

A

Class I

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53
Q

Injury associated with type II hypersensitivity rxns occurs from?

A

IgM and IgG Abs through promotion of phagocytosis or lysis and induction of inflammation

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54
Q

How are immunodeficiencies manifested clinically?

A

increased infections

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55
Q

What protein is critical in the deletion of immature T-cells that recognize peripheral tissue-restricted self-Ags in the thymus?

A

AIRE

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56
Q

Of the forms of HIV, which is most commonly associated with AIDS in the US, Europe and Central Africa?

A

HIV-1

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57
Q

What is the earliest response of an activated CD4+ T-cell?

A

Secretion of IL-2 and expression of high affinity receptors for IL-2

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58
Q

What do patients with hyper-IgM syndrome present with clinically?

A

Recurrent pyrogenic infections

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59
Q

Cytokines produced by Th2 cells

A

IL-4

IL-5

IL-13

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60
Q

Why might a lupus patient have a false positive test result for syphilis?

A

Abs against phospholipid-β2-glycoprotein complex also bind to cardiolipin antigen, which is used in syphilis serology

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61
Q

Secondary antiphospholipid syndrome occurs when?

A

When patients with antiphospholipid antibodies develop venous and arterial thromboses, associated with spontaneous miscarriages and cerebral or ocular ischemia in associated with lupus

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62
Q

GPCRs located on what cell types recognize N-formylmethionyl resides?

A

Neutrophils, macrophages, other leukocytes

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63
Q

Define anergy

A

lymphocytes that recognize self-Ags may be rendered functionally unresponsive

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64
Q

Through what mechanisms can a mother transmit HIV to her infant?

A

In utero through the placenta

During delivery through an infected birth canal

After birth via breast milk

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65
Q

What is characteristic of innate immunity?

A

It is always present

It is mediated by cells and molecules that respond to the products of microbes and dead cells and induce rapid protective host reactions

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66
Q

How is inflammation triggered in type III hypersensitivity reactions?

A

Ab-Ag complexes deposit in tissues, recruit leukocytes which release enzymes and produce ROS

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67
Q

What is the mechanism behind drug reactions?

How do these rxns manifest?

A

The drug alters self proteins, including MHC molecules, and the new Ags are recognized as foreign by T-cells, leading to cytokine production and inflammation.

Manifest as skin rashes

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68
Q

Hyperacute rejection is characterized by

A

marked inflammation and thrombotic microvasculopathy within minutes to hours after transplant 2/2 preformed anti-donor abs (ABO blood group)

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69
Q

What are the clinical manifestations associated with Ataxia telangiectasia

A

Ataxia

Vascular malformation, telangiectasias, often see in the eyes

Immune deficiency, specifically of IgA and IgG leading to respiratory infections, autoimmune diseases and an increased risk of developing CA (lymphomas)

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70
Q

Chediak-Higashi syndrome is an autosomal recessive disorder characterized by

A

defective fusion of the phagosome with the lysosome, therefore not generating the phagolysosome required to kill bacteria

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71
Q

What forms the peptide binding cleft in an MHC II molecule?

A

the α1 portion and β1 portion of the EC aspect of the molecule

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72
Q

Persons with SCID are susceptible to severe infections by what pathogens

A

Candida albicans

Pneumocystis jiroveci

Pseudomonas

cytomegalovirus

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73
Q

What types of Ags are trapped by dendritic cells and MΦ in the spleen?

A

Bloodborne Ags

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74
Q

What antibodies are prsent on all mature, naive B cells and are the Ag-binding component of the B-cell receptor

A

IgM and IgD

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75
Q

Polymorphisms in what gene are associated with Crohn’s disease?

A

NOD2

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76
Q

Among the genes associated with autoimmune diseases, the greatest contribution comes from which ones?

A

HLA genes

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77
Q

What are MHC molecules called in humans?

A

Human leukocyte antigens (HLA)

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78
Q

Why is lymphocyte circulation important to T cells?

A

Naive T cells have to circulate through peripheral lymphoid organs (spleen and LN) where Ags are concentrated

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79
Q

Morphologic changes in the MSK system of patients with sjogren syndrome

A

inflammation of synovium, associated with hypertrophy and hyperplasia of the synovial soft tissues occurs first

Fibrosis develops later without joint destruction

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80
Q

What is the function of macrophages in T-cell activation?

A

They phagocytose microbes, presenting peptide fragments to T-cells

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81
Q

What changes that contribute to autoimmunity are thought to occur as a result of susceptibility genes and environmental triggers?

A

Defective tolerance or regulation

Abnormal display of self-Ags

Inflammation or an intital innate immune response

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82
Q

What cytokine is produced by NK cells?

A

IFN-Υ

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83
Q

What other conditions can develop from human herpesvirus 8?

A

Primary effusion lymphoma

Castleman’s disease

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84
Q

Acute ab-mediated rejection is characterized by

A

Ab production after transplantation

Injury 2/2 complement dependent cytotoxicity, inflammation and ab-mediated cytotoxicity

initial target = graft vasculature

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85
Q
A
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86
Q

What is the final phase of HIV infection characterized by?

A

Progression to AIDS with the breakdown of host defense, a dramatic increase in plasma virus and severe life-threatening clinical disease

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87
Q

What clinical test is essential for the diagnosis of sjogren syndrome?

A

biopsy of the lip to examin minor salivary glands

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88
Q

What immune complexes are found to be the most pathogenic?

A

Those that are medium sized and formed in slight Ag excess

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89
Q

What is characteristic of adaptive immunity?

A

It is a delayed reaction, developing after exposure to microbes and other foreign substances.

It is even more powerful than innate immunity in combating infections

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90
Q

What population is more likely to develop systemic sclerosis?

A

African american women

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91
Q

what are the most potent vasoactive and spasmogenic agents known?

A

Leukotrienes C4 and D4

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92
Q

Tissue injury resulting from a type IV hypersensitivity rxn occurs from what cells?

A

T-cells

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93
Q

Ab-mediated inflammation occurs through what mechanism?

A

Activation of complement

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94
Q

What category of immunity is the first line of defense?

A

Innate immunity

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95
Q

Describe the direct pathway of allorecognition

A

T cells from the transplant recipient recognize the allogeneic (donor) MHC molecules as foreign and mount an immune response against it (with CD8 and CD4 T-cells) leading to damage to renal tubule by inflammation and death of graft cells

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96
Q

T-cells activate both B-cells and macrophages through what cell surface molecules?

A

CD40L on the T-cell

CD40 on the B-cell or MΦ

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97
Q

What autoantibodies are involved in SLE?

A

Antinuclear antibodies (ANAs)

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98
Q

What inhibitory receptors can send inhibtory signals to T-cells that are recognizing self-Ags?

A

CTLA-4

PD-1

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99
Q

What is the association between ankylosing spondylitis and HLA-B27?

A

Individuals who inherit this HLA-B27 allele have a 100-200 fold higher chance of developing the disease

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100
Q

What are the sites of continuous HIV replication and cell destruction during the late phase of the disease?

A

lymph nodes

spleen

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101
Q

What type of helper T-cell is involved in the isotype switching and affinity maturation that occurs within lymphoid organs?

A

Follicular helper T-cells (TFH)

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102
Q

A mutation in Foxp3 in humans results in what systemic autoimmune disease?

A

IPEX (immune dysregulation polyendocrinopathy, enteropathy, X-linked)

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103
Q

Describe the indirect pathway of allorecognition

A

The host dendritic cell recognizes the graft as foreign, uptakes it as an EC Ag, processing it and presenting it to host CD4 cells.

CD4 cells induce delayed type hypersensitivity inflammatory rxn

CD4 cells also help to activate B-cells to produce Abs against graft Ag

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104
Q

Why is the chronic phase of HIV termed the clinical latency period?

A

There are no clinical manifestations of the infection during this time

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105
Q

Treatment for hyper-IgM syndrome

A

IVIg

Stem cell transplant

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106
Q

Regulatory T-cells also express CTLA-4, what is the purpose of this receptor?

A

Bind B7 molecules (CD80/CD86) on APCs, reducing their ability to activate T-cells

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107
Q

In addition to fibrosis of skin and other organs, what other clincial manifestations are seen with systemic sclerosis (scleroderma)?

A

GERD

Esophageal ulceration

Renal vascular disease

Pulmonary HTN

Pulmonary fibrosis

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108
Q

What cell surface molecules are present on NK cells?

A

CD16

CD56

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109
Q

AIDS is characterized by

A

Profound immunosuppression that leads to:

Opportunistic infections

Secondary neoplasms

Neurological manifestations

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110
Q

What are complications seen in patients who are taking immunosuppressive drugs s/p transplantation?

A

Increased susceptibility to opportunistic infections, most commonly polyoma virus

Increased risk of developing EBV-induced lymphomas, HPV induced squamous carcinomas, and kaposi sarcoma

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111
Q

What is present in the bone marrow or in other organs and strongly indicative of SLE?

A

LE cells or hematoxylin bodies

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112
Q

What is the most important mast-cell derived amine?

A

Histamine

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113
Q

Pharyngeal tonsils and Peyer’s patches are examples of what kind of lymphoid tissues?

A

Mucosal

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114
Q

IgG4-related disease is characterized by

A

a constellation of disorders with tissue infiltrates dominated by IgG4 Ab-producing plasma cells, T-lymphocytes, and fibrosis

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115
Q

What Ab acts as an opsonin?

A

IgG

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116
Q

Systemic anaphylaxis is characterized by

A

vascular shock, widespread edema, difficulty breathing

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117
Q

A patient is likely to develop a drug-induced lupus after receiving hydralazine if they have what allele type?

A

HLA-DR6

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118
Q

Amyloidosis is a condition characterized by the deposition of fibrillar proteins that cause tissue damage, what causes the formation of the fibrils?

A

Aggregation of misfolded proteins

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119
Q

What are the structural components share among related microbes, are essential for infectivity, and are recognized by cells of the innate immune response?

A

Pathogen-associated molecular patterns (PAMPs)

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120
Q

X-linked agammaglobinemia is characterized by

A

failure of B-cell precursors to develop into mature B-cells

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121
Q

What does the viral load at the end of the acute phase reflect?

A

The equilibrium reached between the virus and the host response; this is the viral set point and it is the predictor of the rate of decline of Cd4+ T-cells

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122
Q

Autoimmune disorders arise as a result of

A

failure of tolerance to self Ags

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123
Q

What cardiovascular condition is seen in young patients with SLE who have been treated previously with corticosteroids?

A

coronary artery disease

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124
Q

What ethncities have a 2 to 3 fold higher prevalence of SLE than caucasians?

A

African americans

Hispanics

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125
Q

What are the functions that have been attributed to innate lymphoid cells?

A

Early defense against infection

Recognition and elimination of stressed cells

Shaping the adaptive immune response by providing cytokines that influence T-cell differentiation

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126
Q

Autoantibodies specific for RBCs, WBCs, and platelets ____ these cells, promoting phagocytosis and lysis

A

Opsonize

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127
Q

Function of interferon regulatory factors

A

Stimulation of the production of antiviral cytokines, type I interferons

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128
Q

Type I hypersensitivity reactions are often termed allergic reactions, what triggers these reactions?

A

The binding of an Ag (allergen) to an IgE Ab

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129
Q

Patterns of nuclear fluorescence

A

Homogenous or diffuse nuclear staining

Rim or peripheral staining

Nucleolar pattern

Centromeric pattern

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130
Q

What are the best defined regulatory T-cells?

A

CD4+ T-cells with high expression of CD25 and Foxp3

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131
Q

If amyloidosis occurs in the kidney, what occurs?

A

Disruption of glomeruli which can lead to proteinuria and eventually edema

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132
Q

What effect do the preformed enzyme mediators have in an immediate hypersensitivity reaction?

A

They cause tissue damage and lead to the generation of kinins and activated components of complement

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133
Q

What is the most common cause of autosomal recessive SCID?

A

A deficiency in the enzyme adenosine deaminase

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134
Q

Describe the structure of the HIV virion

A

Spherical

Electron-dense, cone-shaped core surrounded by a lipid envelope derived from the host cell membrane

Glycoproteins coat the virion (gp120, gp41)

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135
Q

Injury associated with Type I hypersensitivity rxns is caused by what?

A

Th2 cells

IgE Abs

Mast cells

other leukocytes

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136
Q

Where are pattern recognition receptors located?

A

in cellular compartments where microbes may be present

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137
Q

Susceptibility to immediate hypersensitivity rxns is ______ determined.

A

Genetically

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138
Q

What drugs have been found to induce an SLE-like response in humans?

A

Hydralazine, procainamide and D-penicillamine

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139
Q

Describe the contents of the HIV virion

A

Core contains:

major capsid protein p24

viral enzymes including protease, reverse transcriptase and integrase

HIV-1 RNA genome including gag, pol, env retroviral genes

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140
Q

What type of virus is HIV?

A

Retrovirus that is part of the lentivirus family (slow virus)

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141
Q

What is the mechanism behind the transmission of HIV?

A

Breaching the mucosal barriers introduces the virsu to the bloodstream or infects mucosal dendritic cells

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142
Q

What is the most common secondary immunodeficiency?

A

AIDS

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143
Q

What other proteins are commonly found on T-cells?

A

CD4, CD8, CD28

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144
Q

Cell-mediated (cellular) immunity is mediated by what cell types?

A

T-lymphocytes (T cells)

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145
Q

What chemokine is responsible for drawing eosinophils to the site of immediate hypersensitivity?

A

Eotaxin

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146
Q

where are Υδ T-cell receptors commonly found?

A

epithelial surfaces, including skin and mucosa of the GI tract and urogential tract

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147
Q

What is a common clinical example of a delayed-type hypersensitivity rxn (type IV)?

A

Tuberculin rxn

(PPD test)

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148
Q

Antiphopholipid antibodies are present in 30-40% of lupus patients, what are these Abs directed against?

A

Epitopes of plasma proteins that are revealed when the proteins are in complex with phospholipids

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149
Q

Wiskott Aldrich syndrome is an X-linked disease caused by a mutation in what gene?

A

WASP

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150
Q

What is the function of peripheral lymphoid organs?

A

Concentrate Ags, APCs, and lymphocytes in order to optimize interactions and develop the adaptive immune response

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151
Q

Most common COD in patients with SLE?

A

renal failure

intercurrent infections

152
Q

What are the three major populations of T-cells

A

Helper T cells

Cytotoxic T cells

Regulatory T cells

153
Q

Severe combined immunodeficiency (SCID) represents a constellation of syndromes that all have common defects in

A

humoral and cell-mediate immune responses (both B-cell and T-cell)

154
Q

During T-cell activation, some APCs produce IL-12, which signals the differentiation of CD4+ T-cells into what subset?

A

Th1

155
Q

Explain the process of negative selection that occurs during central tolerance of developing T-cells

A

Some T-cells express TCRs that have high affinity for self-Ags, when they encounter self-Ags in the thymus, these T-cells die by apoptosis to rid these cells from the T-cell pool

156
Q

Most autoimmune disorders are considered to be what type of genetic disorder?

A

complex multigenic

157
Q

What is the function of MHC molecules?

A

Display peptide fragments of protein Ags for recognition by Ag-specific T-cells

158
Q

Morphologic changes of the kidney in patients with SLE

A

Deposition of immune complexes along the basement membrane and glomerulus

159
Q

How do infections trigger autoimmune reactions?

A

Upregulation of costimulators on APCs

Some microbes may express Ags that have the same AA sequence as self Ags (molecular mimicry)

160
Q

What is seen in drug-induced lupus erythematous that is also seen in SLE?

A

Arthralgias

Fever

Positive ANA

Discoid rash

Hematologic disease

Positive immunofluorescence

161
Q

What type of mutation in NOD-like receptors leads to fever syndromes known as autoinflammatory syndromes?

A

Gain-of-function mutation

162
Q

If a B cell has responded to an Ag within the lymph node, what is present within the follicle?

A

Germinal center

163
Q

What antibody, directed against DNA topoisomerase I, is highly specific for systemic sclerosis and is found in immunofluorescent speckled staining?

A

anti-Scl 70

164
Q

Typical presentation of a patient with SLE

A

young female with some, but not necessarily all of these features:

butterfly rash, fever, joint pain, pleuritic CP, photosensitivity

165
Q

What mechanisms are involved in peripheral tolerance?

A

Anergy

Suppression by regulatory T-cells

Deletion by apoptosis

166
Q

What cytokine is the most potent eosinophil activator known?

What is the source of this cytokine?

A

IL-5

Th2 cells

167
Q

What signals are released from injured or necrotic cells and are recognized by leukocytes?

A

Damage-associated molecular patterns (DAMPs)

168
Q

What is responsible for the depletion of cells coated with abs?

A

Phagocytosis

169
Q

Morphologic changes in the skin of patients with sjogren syndrome

A

diffuse sclerotic atrophy of skin, beginning in the fingers (sclerodactyly) and extending proximally up the arms toward the neck and face

170
Q

What is the mechanism behind the late-phase rxn of a type I hypersensitivity rxn?

A

The immediate rxn induces the release of various cytokines and chemokines, including TNF and IL-1, these cytokines recruit leukocytes which act to sustain the inflammatory response without further exposure to the Ag

171
Q

What cells are the only ones in the body capable of producing antibodies?

A

B lymphocytes

172
Q

Various protein Ags that form immune complexes and result in serum sickness lead to what clinicopathologic manifestations?

A

Arthritis

Vasculitis

Nephritis

173
Q

Where are naive T lymphocytes activated and where do they end up?

A

In peripheral lymphoid organs

Anywhere that the Ag is present

174
Q

Release of what transcription factor leads to the viral transcription of HIV through LTR?

A

NF-kB

175
Q

MHC I molecules are made up of a polymorphic α chain and a nonpolymorphic β2-microglobulin. The α chains are encoded by three genes, what are they?

A

HLA-A

HLA-B

HLA-C

176
Q

What proteins are critical for HIV infection of host cells by helping with attachment?

A

gp120

gp41

177
Q

Morphologic changes in the kidneys of patients with sjogren syndrome

A

Vascular lesions

Thickening and fibrinous deposition in aa

Fibrinoid necrosis can develop in patients with HTN

178
Q

What proteins do eosinophils contain that induce tissue damage?

A

Major basic protein

eosinophil cationic protein

179
Q

What is a major barrier to transplantation?

A

Rejection: recipient’s immune system recognizes the graft as foreign and attacks it (host vs graft disease)

180
Q

Morphologic changes seen in the skin of patients with SLE

A

Butterfly rash (50% of patients) along the bridge of the nose and cheeks

Urticaria, bullae, or maculopapular lesions can develop

Histologically, there is vacuolar degeneration of the epidermis, vasculitis with fibrinoid necrosis

181
Q

Pathogenesis of systemic immune complex disease occurs in three stages:

A
  1. formation of immune complexes
  2. deposition of immune complexes
  3. inflammation and tissue injury
182
Q

What are the principle costimulators for T cells and what do they bind?

A

B7 proteins (CD80 and CD86)

Bind CD28 on T cells

183
Q

What clinical measurement is used to determine when to start antiretroviral therapy in patients with HIV?

A

serum CD4+ counts

184
Q

What is the fundamental defect in SLE?

A

Failure of the mechanisms that maintain self-tolerance

185
Q

Cells involved in adaptive immunity?

A

Lymphocytes

Antibodies

186
Q

Polysaccharides and lipids stimulate the secretion of what Ab?

A

IgM

187
Q

What HIV gene encodes the viral enzymes?

A
  • Pol*
  • (polymerase)*
188
Q

Function of helper T cells

A

Stimulate B lymphocytes to make Abs and activate other leukocytes to destroy microbes

189
Q

The B-cells of lymphomas seen in AIDS patients are commonly infected with what virus?

A

EBV

190
Q

A patient is likely to develop a drug-induced lupus after receiving procainamide if they have what allele type?

A

HLA-DR4

191
Q

NK cell inhibitory receptors recognize what molecules and help to prevent what from happening?

A

They recognize self class I MHC molecules and help to prevent NK cells from killing normal cells

192
Q

Sjogren disease is caused by

A

immunologically mediated destruction of lacrimal and salivary glands followed by fibrotic destruction

193
Q

What are the three forms of ab-mediated rxns seen in transplant rejection?

A

Hyperacute rejection

acute ab-mediated rejection

chronic ab-mediated rejection

194
Q

Deficiency in DAF or CD59 will result in what disease?

A

Paroxysmal nocturnal hemoglobinuria

195
Q

Morphologic changes in the spleen seen in patients with SLE

A

Splenomegaly

capular thickening

follicular hyperplasia

196
Q

What is the onset of SLE?

A

Acute or insidious

197
Q

In lymph nodes, where are the B cells and T cells located

A

B cells are located in follicles along the periphery of the LN

T cells are located in the paracortex of the LN, next to the follicles

198
Q

What molecules do the Υδ TCR recognize?

Does this receptor complex require MHC display?

A

Peptides, lipids, small molecules

No

199
Q

DiGeorge syndrome occurs 2/2

A

Failure of the development of the 3rd and 4th pharyngeal pouches, leading to defects in the thymus, parathyroid glands, heart and great vessels

200
Q

Chronic granulomatous disease is a group of genetic disorders characterized by

A

defects in superoxide production within phagocytes, resulting in subsequent defects in bacterial killing, rendering the patients susceptible to recurrent bacterial infection

201
Q

Humoral immunity is mediated by what cell types?

A

B-lymphocytes (B cells) and their products, Abs

202
Q

Injury is caused by what in SLE?

A

deposition of immune complexes and binding of abs to various cells and tissues

203
Q

What is the function of natural killer cells?

A

to destroy irreversibly stressed and abnormal cells, such as virus-infected cells and tumor cells

204
Q

What is essential in the developement and maintenance of regulatory T-cells?

A

IL-2

Foxp3

205
Q

Type III hypersensitivity reactions are characterized by

A

formation of Ag-Ab immune complexes that deposit into vessel walls, causing inflammation at these sites

(can also deposit in tissues)

206
Q

What determines if a lymphocyte is mature or naive?

A

Whether or not it was encountered a specific Ag

207
Q

The HLA system is highly polymorphic, why is this problematic?

A

There are many alleles of MHC genes and each individuals alleles differ, therefore a berrier in organ transplantation exists

(i.e. rejection)

208
Q

What two problems are unique to hematopoietic stem cell transplantation?

A

graft vs host disease

immunodeficiency

209
Q

Cytokine produced by Th1 cells

A

IFN-Υ

210
Q

How does innate immunity differ from adaptive immunity?

A

Innate immunity does not have memory or fine Ag specificity

211
Q

How is the graft, vs host disease minimized in hematopoietic stem cell transplants?

A

The donor and recipient are HLA matched prior to transplantation

212
Q

What is the function of a regulatory T-cell?

A

Prevent immune rxns against self-Ags

213
Q

Why do some patients with IgA defiency develop anaphylaxis after receiving blood transfusion?

A

IgA behaves like a foreign antigen and patients’ immune systems develop immune rxn to it

214
Q

Why are antiphospholipid antibodies sometimes considered lupus anticoagulants?

A

They interfere with clotting tests, such as aPTT

215
Q

Lymphocytes constantly circulate between tissues and particular sites, where do naive lymphocytes travel to?

A

They traverse lymphoid organs where immune responses are initiated

216
Q

Where are class I MHC molecules located

A

All nucleated cells

Platelets

217
Q

What are common complications that can be seen in patients with Sjogren syndrome?

A

Pulmonary fibrosis

lymphoma 2/2 B-cell proliferation becoming clonal

218
Q

X-linked agammaglobinemia is caused by mutations in what gene?

A

The Bruton tyrosine kinase gene on the X chromosome

219
Q

How is antigen receptor diversity accomplished?

A

Somatic recombination of genes that encode receptor proteins

220
Q

What triad is characteristic of Wiskott aldrich syndrome?

A

Thrombocytopenia

Eczema

Recurrent infections

221
Q

Function of caspase-1

A

Cleaves precursor form of IL-1, generating the biologically active form

222
Q

How do dendritic cells resemble macrophages and how are they different?

A

They initiate innate immune responses, but unlike macrophages, they are not key participants in eliminating the offending agent

223
Q

Morphologic changes in the alimentary tract of patients with sjogren syndrome

A

Atrophy and collagenous fibrosis of musclaris, most severely in the esophagus

GERD

Barrett metaplasia

Strictures

Loss of villi and microvilli leading to malabsorption syndrome

224
Q

Patients with CVID often present with

A

Recurrent sinopulmonary pyogenic infections

Granulomas

Chronic diarrhea 2/2 Giardia lamblia

Autoimmune diseases

225
Q

Infection of what cells are the foundation of the neuropathic effects of HIV?

A

microglia

226
Q

Where are the genes encoding HLA located?

A

On chromosome 6

227
Q

Function of cell-mediated immunity?

A

Defense against intracellular microbes

228
Q

Type II hypersensitivity reactions occur when

A

Abs react with Ags present on cell surfaces or in ECM, causing the destruction of cells followed by inflammation OR by interfering with the normal function of cells

229
Q

Sjogren disease is characterized by

A

dry eyes

dry mouth (xerostomia)

230
Q

What is the purpose of a memory cell?

A

React rapidly and strongly to combat a microbe it has already been exposed to, if it returns

231
Q

What is the molecular composition of an MHC class II molecule?

A

a heterodimer with one α chain and one β chain

232
Q

Function of NF-kB

A

Stimulates the synthesis and secretion of cytokines and the expression of adhesion molecules

233
Q

What peptide inserts into the cell membrane of the target cell, leading to the fusion of the HIV to the host?

A

gp41

234
Q

Mast cells and basophils involved in the immediate hypersensitivity rxns have what cell surface receptor?

What does this receptor bind?

A

FcεRI

Fc portion of IgE

235
Q

How many receptors does innate immunity use?

What about adaptive immunity?

A

100

2

236
Q

What tx options are available for SCID?

A

Hematopoietic stem cell transplant

Gene therapy

237
Q

What is the most common neoplasm in AIDS patients?

A

Kaposi sarcoma

238
Q

Because IgA is the major Ab in external secretions, mucosal defenses are weakend. Infections thus commonly occur where?

A

Respiratory, GI and GU tracts

239
Q

What virus is associated with the development of kaposi sarcoma

A

Human herpesvirus 8

240
Q

What is the most important Ag-presenting cell for initating T-cell responses against Ags?

A

Dendritic cells

241
Q

What constitutes the initial step in infection by HIV?

A

Binding of the surface glycoprotein gp120 to CD4 receptor molecule, which leads to a conformational change that allows for a new recognition site on gp120 for coreceptors (CCR5, CXCR4)

242
Q

What are granzymes and what is their function in CTL mediated cell killing?

A

Granzymes are proteases that cleave and activate caspses which induce apoptosis of the target cell

243
Q

Where is CR2/CD21 located and what is its function?

A

On B-cells

Recognizes complement products generated during the innate immune response

244
Q

What Ags do MHC II molecules present?

A

Those that are internalized into vesicles, derived from extracellular microbes and soluble proteins

245
Q

Where is CD40 located and what is its function?

A

On B-cells

Receives signals from helper T-cells

246
Q

What are the peripheral lymphoid organs?

A

Spleen

Lymph nodes

mucosal and cutaneous lymphoid tissues

247
Q

Chronic ab-mediated rejection is characterized by

A

fibrosis with primary effect on vasculature

248
Q

What HIV gene is responsible for encoding the surface glycoproteins, gp120 and gp41, important for HIV infection?

A
  • env*
  • (envelope protein)*
249
Q

Th1 cells secrete what cytokine, which promotes further Th1 cell development and thus amplifies the rxn?

A

IFN-Υ

250
Q

When a lymphocyte differentiates into an effector cell, what is its function?

A

eliminating microbe for which it is specific for

251
Q

What is the function of IL-2 in respect to T-cells?

A

IL-2 stimulates proliferation of T lymphocytes

252
Q

What is the most abundant mediator formed in mast cells by the cyclooxygenase pathway?

A

Prostaglandin D2

253
Q

In immunizations, adjuvants are given with the Ag in or do stimulate an innate immune response, what is the purpose of the adjuvant?

A

To activate APCs to express costimulators and to secrete cytokines that will stimulate the proliferation and differentiation of T lymphocytes

254
Q

What are the three most common forms of amyloid?

A

Amyloid light chain

Amyloid-associated type

Beta-amyloid protein

255
Q

What results from inherited defects in RAG proteins?

A

failure to generate mature lymphocytes

256
Q

The most common form of SCID follows what inheritance pattern and hence is more common in?

A

X-linked

More common in boys

257
Q

ataxia telangiectasia is an autosomal recessive disorder caused by a mutation in what gene, leading to a defect in?

A

ATM gene

Defective DNA repair

258
Q

What does innate immunity provide that stimulates the adaptive immune response?

A

Danger signals

259
Q

What are the 5 groups of adults who are high risk of developing AIDS?

A

Homosexual or bisexual men

IVDA

Hemophiliacs

Heterosexual contacts of members of other high risk groups

Newborns in areas of high female prevalence

260
Q

Antinuclear antibodies are grouped into four cateogories, what are they?

A

Abs to DNA

Abs to histones

Abs to nonhistone proteins bound to RNA

Abs to nucleolar ags

261
Q

Two categories of mechanisms of defence against microbes

A

Innate immunity

Adaptive immunity

262
Q

What is the most abundant viral Ag that is detected via ELISA and used to diagnose HIV infection?

A

p24

263
Q

What cytokine is produced by CD8+ T-cells and contributes to inflammatory rxns seen in delayed type hypersensitivity?

A

IFN-Υ

264
Q

What are the common immune-privileged sites?

Why are the immune privileged?

A

Testis, eye, brain

It is difficult to induce immune response to Ags introduced into these sites

265
Q

What cytosolic receptor recognizes products of necrotic cells, ion disturbances, and some microbial products?

A

NOD-like receptors

266
Q

Mixed CT disease is characterized by high seriology titers of what antibody?

A

Anti-ribonucleoprotein (anti-RNP)

267
Q

What cytokines are involved in the regulation of NK cell activity?

A

IL-2

IL-15

IL-12

268
Q

How do B cells respond when they recognize self-Ags?

A

They undergo receptor editing, where antigen receptors undergo gene rearrangment to generate new Ag receptors not specific to self-Ags

269
Q

How can CREST syndrome be identified using immunofluorescence?

A

Staining is centromeric, staining for anticentromere abs

270
Q

Chronic discoid lupus erythematosus is characterized by

A

presence of skin plaques showing varying degrees of edema, erythema, scaliness, follicular plugging, and skin atrophy surrounded by an elevated erythematous border

271
Q

systemic sclerosis (scleoderma) is characterized by

A

Chronic inflammation though to be a result of autoimmunity

Widespread damage to small blood vessels

Progressive interstitial and perivascular fibrosis in the skin and multiple organs

272
Q

Class II MHC molecules are encoded in what region?

A

HLA-D

273
Q

What is the most common form of nephritis associated with SLE?

What is the least common form?

A

Class IV

Class I

274
Q

Type IV hypersensitivity reactions are mediated by T-cells and result in injury how?

A

inflammation 2/2 cytokines produced by CD4+ T cells

OR

Cell killing by CD8+ T-cells

275
Q

What are the classic primary adaptive immunodeficiencies associated with lymphocyte activation or function?

A

Hyper-IgM syndrome

Common Variable Immunodeficiency (CVID)

IgA deficiency

276
Q

What is the major antigenic difference between donor and recipient, resulting in transplant rejection

A

differences in HLA alleles

277
Q

When abs deposit in fixed tissues, such as the basement membrane or ECM, the resultant injury is due to what?

A

Inflammation

278
Q

Purpose of immunity

A

Protection from infectious pathogens

279
Q

Function of humoral immunity

A

Protect against extracellular microbes and their toxins

280
Q

Mannose receptor function

A

Recognize microbial sugars and infuce phagocytosis of these microbes

281
Q

What type of peptides are displayed by MHC I molecules?

A

Peptides derived from proteins of viral and tumor Ags which are located in the cytoplasm and usally produced in the cells

282
Q

What type of immune reaction causes injury?

A

Hypersensitivity rxns

283
Q

Cause of primary immunodeficiencies

A

genetics

284
Q

Two types of adaptive immunity

A

Humoral immunity

Cell-mediated immunity

285
Q

AIDS is caused by

A

Retrovirus human immunodeficiency virus (HIV)

286
Q

What is the principal mechanism of T-cell mediated killing of targets (via CD8+ CTLs)?

A

CTLs release a complex of perforins and granzymes, perforin generates a hole in the target cell allowing for granzymes to enter

287
Q

What are the principle generative lymphoid organs?

A

Thymus

Bone marrow

288
Q

HLA-D has three subregions, what are they?

A

HLA-DP

HLA-DQ

HLA-DR

289
Q

What are the major lipid mediators of an immediate hypersensitivity rxn?

A

Arachidonic acid derived products including PGD2, LTB4, LTC4, LTD4

290
Q

Common treatment for autoinflammatory syndromes?

A

IL-1 antagonist

291
Q

What are the serologic markers for sjogren syndrome?

A

anti-RO/SS-A and Ati-La/SS-B

292
Q

What opportunistic infections are commonly seen in AIDS patients?

A

Pneumocystis jiroveci

Cytomegalovirus

Tuberculosis

Toxoplasma

Candidiasis

293
Q

Function of regulatory T cells

A

Limit immune responses and prevent rxns against self-Ags

294
Q

What are the coreceptors in T-cell activation, as they recognize the same ligand that the Ag receptor sees?

A

CD4 and CD8

295
Q

Common variable immunodeficiency is a group of disorders with a common feature of

A

Hypogammaglobulinemia, usually affecting all ab classes but sometimes just IgG

296
Q

What is the hallmark of SLE?

A

Production of antibodies

297
Q

Morphologic changes in the lungs of patients with sjogren syndrome

A

Pulmonary HTN

Pulmonary fibrosis

298
Q

What makes up the T-cell receptors?

A

disulfide-linked heterodimer made up of an α and β chain, each having a variable (Ag-binding) region and a constant region

299
Q

Where does isotype switching and affinity maturation occur?

A

In germinal centers of lymphoid follicles

300
Q

What has been found to be the cause of many chronic inflammatory diseases?

A

CD4+ T cell-mediated hypersensitivity rxns induced by environmental and self Ags

301
Q

Clinical manifestations of CVID are caused by

A

antibody deficiency

302
Q

What cell type is central in the development of immediate hypersensitivity?

A

Mast cells

303
Q

The cause of systemic sclerosis is unknown, but is thought to arise from 3 interrelated processes, what are they?

A

Autoimmune responses

Vascular damage

Collagen deposition (fibrosis)

304
Q

The EC region of the α chain of MHC I molecule has three domains, α1 α2 and α3.

α1 and α2 form a cleft, what binds in this cleft?

A

Peptides

305
Q

What are the three requirements in order for a disorder to be categorized as pathologic autoimmunity?

A

Presense of an immune rxn specific for some self antigen or self tissue

Evidence that such a rxn is not 2/2 tissue damage but is of primary pathogenic significance

The absense of another well-defined cause of disease

306
Q

The immediate rxn of type I hypersensitivity recations is characterized by

A

Vasodilation

vascular leakage

smooth muscle spasm OR glandular secretion (location dependent)

307
Q

Define epitope spreading

A

An immune response against one self-Ag causes tissue damage, releasing other ags and resulting in the activation of lymphocytes by these newly encountered epitopes

308
Q

Immature dendritic cells of the epidermis

A

Langerhans cells

309
Q

What amyloid type constitutes the core of cerebral plaques found in Alzheimer disease?

A

beta-amyloid

310
Q

Function of CD16 on NK cells?

A

Allows NK cells to lyse IgG-coated target cells

311
Q

Hyper-IgM syndrome is caused by mutations in a gene encoding what protein?

A

CD40L

312
Q

What is the acute retroviral syndrome?

A

The clinical presentation of the intial spread of the virus and the host response; characterized by fever, sore throat, myalgias

313
Q

If amyloid occurs in the heart, what occurs?

A

Disruption of the myocardium leading to dysrhythmias

314
Q

What is a possible first indicartion of IgA deficiency?

A

Anaphylaxis s/p blood transfusion

315
Q

The systemic lesions seen in SLE are caused by

A

immune complexes (type III hypersensitivity rxn)

316
Q

How do NOD-like receptors signal?

A

Via the inflammasome, which activates caspase-1

317
Q

The enzyme responsible for recombination of gene segments in developing lymphocytes is the product of what genes?

A

RAG-1 and RAG-2

318
Q

Lymphocytes constantly circulate between tissues and particular sites, where do effector lymphocytes travel to?

A

To sites of infection and inflammation

319
Q

Morphologic changes of blood vessels in patients with SLE

A

acute necrotizing vasculitis

fibrinoid deposits in vessel walls

320
Q

What are the two mechanisms of self-tolerance?

A

Central tolerance

Peripheral tolerance

321
Q

What method is used to detected antinuclear antibodies?

A

indirect immunofluorescence

322
Q

PAMPs and DAMPs collectively form a group of molecules that are termed

A

Pattern recognition receptors (PRRs)

323
Q

Morphologic changes seen in delayed type hypersensitivity include

A

accumulation of mononuclear cells, mainly CD4+ T-cells, and macrophages around venules, producing a “cuffing”

324
Q

Examples of disorders where abs are directed against cell surface receptors, causing a dysregulation in normal function without cell injury or inflammation

A

Graves disease

Myasthenia gravis

325
Q

Inherited defects in leukocyte adhesions is considered a defect in innate immunity and manisfests with

A

Recurrent bacterial infections d/t inadequate granulocyte function

326
Q

What are the two major targest of HIV infection?

A

immune system

CNS

327
Q

What HIV gene is responsible for encoding the proteins inside the virus?

A

gag

328
Q

Define self-tolerance

A

Lack of responsiveness to your own antigens

329
Q

After stimulation by Ag, B-cells develop into what cell type?

A

Plasma cells, which are veritable protein factories for Abs

330
Q

What additional lipid mediator is produced by mast cells, causes platelet aggregation, release of histamine, bronchospasm, increased vascular permeability and vasodilation?

A

PAF (platelet activating factor)

331
Q

What is typically present in 70% of patients with systemic sclerosis, often manifesting prior to other sxs?

A

Raynaud’s phenomenon

332
Q

Hyper-IgM syndrome is characterized by

A

inability to class switch d/t the lack of CD40L

333
Q

In class IV nephritis associated with SLE, patients develop lesions in half or more of their glomeruli. These patients often are symptomatic, showing what symptoms?

A

Hematuria

Proteinuria

HTN

Renal insufficiency

334
Q

Sexual transmission of HIV is enchanced by what?

A

Coexisiting STIs

335
Q

What are the tree major routes of HIV transmission?

A

Sexually

Parenteral inoculation

Passage from mother to their newborns

336
Q

What is the best known pattern recognition receptor?

A

toll-like receptor (TLR)

337
Q

What cells are located within germinal centers of lymphoid follicles in the spleen and lymph nodes that trap Ag bound to Abs or complement proteins

A

Follicular dendritic cells

338
Q

Where do T cells mature?

A

In the thymus

339
Q

Where do B cells mature

A

In the bone marrow

340
Q

If an APC produces IL-1, IL-6 and IL-23, the CD4+ T-cell is stimulated to differentiate into what subtype?

A

Th17

341
Q

The late-phase rxn of a type I hypersensitivity reaction is characterized by

A

an onset of 2-24 hours after exposure

infiltration of tissues with eosinophils, neutrophils, basophils, monocytes, CD4+ T-cells and tissue injury

342
Q

Mixed connective tissue disease is a combination of what disorders?

A

SLE

Scleroderma

Polymyositis

343
Q

Injury to what regions of the body is prominent in SLE?

A

Skin, joints, kidney, serous membranes

344
Q

What is a common presenting feature of patients with mixed CT disease?

A

Raynaud’s phenomenon

345
Q

Deficiency in the C1 inhibitor leads to what condition?

A

Hereditary angioedema

346
Q

what types of dendritic cells are important for the initiation and maintanence of HIV infection?

A

Mucosal

Follicular

347
Q

The TCR is covalently linked to 6 polypeptide chains, forming the CD3 complex and ζ chain dimer, what is the function of these?

A

Transduction of signals into the T cell that are triggered by Ag binding to TCR

348
Q

What profound B-cell abnormalities are seen in patients with HIV?

A

Polyclonal activation of B-cells leading to germinal center hyperplasia and possible development of lymphoma

Hypergammaglobulinemia

Reduced isotype switching

Reduced Ab production

349
Q

Define immunologic tolerance

A

the phenomenon of unresponsivenss to an Ag induced by exposure of lymphocytes to that Ag

350
Q

subacute cutaneous lupus erythematosus has a strong association with what antibodies and what genotype?

A

Ab to SS-A Ag

HLD-DR3 genotype

351
Q

Morphologically, what defines a natural killer cell?

A

Larger in size

Azurophilic granules

352
Q

What immunosuppressive drugs are commonly used to increase graft survival after transplantation?

A

Steroids

Tacrolimus (to inhibit T cell function)

353
Q

Hypersensitivity reactions can be triggered by what types of Ags?

A

Endogenous self-Ag

Exogenous environmental Ags

354
Q

X-linked lymphoproliferative syndrome is characterized by

A

the inability to eliminate Epstein-Barr virus (EBV), eventually leading to infectious mono and development of B-cell tumors

355
Q

Tx for Wiskott-Aldrich syndrome

A

stem cell transplant

356
Q

Function of CD8+ T-cells

A

They function as cytotoxic T-cells (CTLs), destroying host cells that are harboring microbes

357
Q

What do TCRs recognize?

A

Peptide Ags that are presented by major histocompatibility complex (MHC) molecules on the surfaces of Ag-presenting cells

358
Q

CD4 T cells bind what class of MHC molecule?

A

Class II

359
Q

Because of the impaired humoral (B-cell) immunity, patients with HIV are susceptible to infection by what types of organisms?

A

Encapsulated bacteria (S. pneumoniae, H. influenzae)

360
Q

What is the appearance of amyloid on an H&E stain?

A

Amorphous, eosinophilic, hyaline extracellular substance

361
Q

What forms the TCR complex?

A

TCR

CD3

ζ chain

362
Q

Graft vs host disease is mediated by what cell type?

What are the common clinical manifestations of this?

A

T-cell mediated

Skin rash leading to desquamation, jaundice leading to cholestasis, blood diarrhea leading to bowel strictures

363
Q

Ab-mediated cell destruction and phagocytosis occurs in what situations?

A

Transfusion rxns

Hemolytic disease of the newborn (erythroblastosis fetalis)

Autoimmune hemolytic anemia, agranulocytosis and thrombocytopenia

Certain drug rxns

364
Q

How is the activity of NK cells regulated?

A

Through the balance of signals from activating and inhibitory receptors

365
Q

Chronic granulomatous disease attempts to control the bacterial infection through what mechanism, as the normal phagocytic O2-dependent killing is inactive?

A

Activated macrophages wall off the infection, forming granulomas

366
Q

When a toll-like receptor is activated, regardless of the type, a common pathway culminates in the activation of what transcription factors?

A

NF-κB

Interferon regulatory factors (IRFs)

367
Q

What are the two major classes of MHC molecules?

A

Class I

Class II

368
Q

Where are the abnormal amyloid fibrils deposited?

A

Extracellular space in various tissues and organs of the body

369
Q

IgG4-related disease often affects what population?

A

middle-aged and older men

370
Q

Isolated IgA deficiency is characterized by

A

extremely low levels of both serum and secretory IgA

371
Q

What is part of innate immunity, providing protection against inhaled microbes?

A

Lung surfactant

372
Q

What occurs when there is a deficiency in any complement protein involved in the formation of the membrane attack complex (MAC)

A

There is no lysis of microbial membrane

increased susceptibility to Neisseria bacteria

373
Q

Cytokines produced by Th17 cells

A

IL-17

374
Q

Function of cytotoxic T cells

A

kill infected cells

375
Q

What molecules are essential for signal transduction through B-cell Ag receptor

A

Igα (CD79a)

Igβ (CD79b)

376
Q

Patients with IgA deficiency present with

A

recurrent sinopulmonary infections

diarrhea

MOD Exam 2 (5 decks)

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