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MOD Exam II > (2) Diseases of the Immune System II (Singh) > Flashcards

(2) Diseases of the Immune System II (Singh) Flashcards

1
Q

What is allorecognition?

A

The ability of an individual organism to distinguish its own tissues from those of another

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2
Q

What is the direct pathway for Allorecognition?

A

Donor organ carries an APC cell

Donor APC cell makes contact with host immune system

This initiates a targeted attack toward graft

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3
Q

What is the indirect pathway for Allorecognition?

A

Recipient’s APCs investigate the contents of the graft cells

Recipient’s APCs recognize graft as non self and elicit a targeted response against graft

This is a B and T cell response against the graft

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4
Q

Which of the following is B-cell mediated?

Hyperacute rejection

Acute rejection

Acute antibody-mediated rejection

Chronic rejection

Chronic antibody-mediated rejection

A

Hyperacute rejection

Acute antibody-mediated rejection

Chronic antibody-mediated rejection

*Anything with antibody in it is gonna be B cell mediated

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5
Q

What is the mechanism of a hyperacute rejection reaction?

A

Mediated by pre-formed antibodies

Accounts for the extremly quick response! Antibodies already exhist in host to act on graft!

In particular…Blood antibodies (ABO)

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6
Q

What type of antibody mediated rejection is this?

A

Acute Antibody-mediated rejection

*Note: Also do a Complement C4d stain to confirm this is acute antibody-mediated rejection

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7
Q

What type of antibody mediated rejection is this?

A

Chronic antibody-mediated rejection

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8
Q

What is this histologic image revealing?

A

Acute cellular rejection (T-cell mediated)

Tubulitis

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9
Q

Why do we care about the mechanism behind these different types of graft rejection?

A

Treatment is very different!!!

The way you treat a T-lymphocyte mediated cellular rejection is very different from an Antibody-mediated (humoral) rejection

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10
Q

What do we have to remember when we treat a patient with graft rejection?

A

There is no more rejection…

BUT!!!!

There are going to be issues because you induced Immunosuppression

Your patient is more prone to:

  • Infections
  • Tumors
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11
Q

What specific viral infection are immunosuppressed from transplant patients suseptible to?

A

VIRAL = Polyomavirus

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12
Q

What is the major occurance with a hematopoietic stem cell transplant?

A

Brand new immune system!!!

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13
Q

What is graft vs. host disease?

A

T-lymphocyte mediated response of the GRAFT against the host!

Common with bone marrow transplant.

Can occur when graft contains immune cells from donor

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14
Q

What are some common manifestations of graft vs. host disease?

A

Reactions in:

Skin - (rash –> desquamation)

Liver - (jaundice –> cholestasis)

Intestines - (bloody diarrhea –> strictures)

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15
Q

Chediak Higashi Syndrome

Inheritance pattern?

Mechanism?

A

AR

Failure of phagolysosomal function

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16
Q

Is the mechanism of Chediak Higashi syndrome microscopically visualized?

A

YES!

The failure of phagolysosomal fusion can be seen on a peripheral blood smear

*You can see GIANT granules in neutrophils.

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17
Q

What is the major clinical presentation of chediak higashi syndrome?

A

Albanism!!!

Defects in melanocytes lead to albanism or grey hair streaks in Chediak Higashi syndrome pts

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18
Q

Chronic granulomatous disease

How is it aquired?

Mechanism?

A

Genetically

Failure of superoxide production within phagocytes. Accumulation of macrophages“walls off” the infection because the innate immune system is compromised and cannot handle the infection!

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19
Q

Membrane attack complex deficiency

What is affected?

What happens without MAC?

What infections are common?

A

Terminal components C5, 6, 7, 8, 9

Without the final MAC, the lysis of the microbial membrane can’t occur

Neisseria infections, Meningitis

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20
Q

Hereditary angioedema

Genetic inheritance pattern?

Mechanism?

A

AD

Deficiency of C1 inhibitor

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21
Q

What are the symptoms of hereditary angioedema?

A

Facial swelling

Airway constriction

Intestinal swelling

TONS OF SWELLING OVERALL

*Remember, this is NOT an autoimmunity deficiency. It’s randomly here. meh.

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22
Q

Severe Combined Immunodeficiency (SCID)

What are two major facets of this disease?

“Bubble” “Boy”

A

“Bubble” –> Such a severe immunodeficiency, knocking out T and B cell response. Need to be isolated from pathogens

“Boy” –> X-linked, FAR more common in boys

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23
Q

What is the treatment for severe combined imunodeficiency (SCID)?

A

Stem cell transplantation

Gene therapy

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24
Q

DiGeorge Syndrome

What are the primary causes?

A

Primary deficiency of T lymphocytes: due to failure of pharyngeal pouches 3/4, thymus, parathyroids, heart, great vessels are affected

Genetic: 22q11 deletions

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25
Q

What are the clinical manifestations of DiGeorge syndrome?

A
  • Facial and palatal abnormalities
  • Cardiac abnormalities
  • Tetany
  • Immune deficiency
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26
Q

X-Linked Agammaglobulinemia

Aka?

Mechanism?

A

“Bruton’s agammaglobulinemia”

Inability of pre-B cells to mature

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27
Q

Generally, what is the presentation of agammaglobulinemia?

A

Encapsulated bacteria

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28
Q

Hyper-IgM syndrome

What kind of mutation?

What does this cause?

How do you treat?

A

CD40/CD40L mutations

Interference w/ T cells helping B cells to class switch. Increased IgM levels, but lower leverls of other classes.

Treat with IVIg, stem cell transplantation

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29
Q

Common Variable Immunodeficiency (CVID)

A collection of disorders resulting in…

A

Hypogammaglobulinemia

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30
Q

What are some manifestations of Common Variable Immunodeficiency (CVID)?

A

Recurrent infections

Granulomas

Chronic diarrhea (Giardia)

Autoimmune disease

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31
Q

IgA Deficiency (isolated)

Describe:

A

W/out IgA, you have less defense against inhaled and ingested pathogens

Frequent:

Sinus/respiratory infections

Urinary bladder infections

GI infections

Also get:

Autoimmune disease and allergies

***Anaphylactic reaction against red cell transfusion

32
Q

What is potentially a first indication a patient has an IgA deficiency?

A

Transfusion-related anaphylaxis

33
Q

Wiskott Aldrich syndrome

Gene mutation?

A

WASP gene mutation

34
Q

Wiskott Aldrich syndrome

Symptoms?

A

Triad of:

  1. Thrombocytopenia
  2. Eczema
  3. Recurrent infections
35
Q

Wiskott Aldrich Syndrome

Treatment?

A

Stem cell transplant

36
Q

Ataxia Telangiectasia

What kind of disease?

What is a common sign?

Major problems that arise?

Inheritance pattern?

A

Neurodegenerative disease

Vascular malformation (can see in sclera of eyes)

Immune deficiency causes, respiratory infections, autoimmune disease, cancer

AR

37
Q

What can cause secondary immunodeficiency?

A

Cancer

Malnutrition

Metabolic disease

Chronic illness

Treatment-related (chemotherapy, radiation, immunosuppression)

Acquired immunodeficiency syndrome (AIDS)

38
Q

Define:

Aquired Immunodeficiency syndrome (AIDS)

A

AIDS is the manifestation that occurs when the HIV affects the body to a degree where immune dysfunction results in:

  1. Opportunistic infections
  2. Secondary neoplasms
  3. Neurologic manifestations
39
Q

How does HIV spread?

A

Sex w/out a condom

Passed from mother to baby

Sharing injecting equipment

Contaminated blood transfusions and organ transplants

40
Q

Who is at risk for aquiring HIV?

A
  • Homosexual/bisexual men
  • IV drug users
  • Hemophiliacs
  • Other recipients of blood/blood components
  • Heterosexual contacts of the above groups
  • Newborns in areas with high female prevalence
41
Q

HIV Transmission via transfusions

What are some important things to note with this mode of transmission?

A

Health care professionals do test blood products before administering…BUT

No test is perfect

There is a window of time from exposure to a positive test that gives the opportunity for a “false negative” test

42
Q

HIV transmission from mother to child

What are the ways this can occur?

A

In utero through placental –> fetal transfer of virus

During delivery w/ contact of secretions in the birth canal

After birth with ingestion of breast milk

43
Q

What is the likelihood that you will get infected with….

HIV

Hepatitis C

following a needle stick accident

A

HIV = 0.3%

Hepatitis C = 30%

44
Q

What is the classification of HIV?

A

Retrovirus

Lentivirus family

45
Q

HIV structure and Content

What is an important capsid protein?

Why do we care?

A

p24

We can test for p24 antigen

46
Q

HIV structure and Content

What are important glycoproteins?

Why do we care?

A

gp120 and gp41

Important for attachment

GOOD DRUG/VACCINE TARGETS

47
Q

HIV structure and Content

What are the important viral enzymes?

A

Protease

Reverse transcriptase

Integrase

48
Q

There are 4 high yield regions Dr. Singh wants us to know about in the HIV-1 genome. What are they?

A

LTR

gag

env

pol

49
Q

What is the function of each region listed below in HIV-1?

LTR

gag

env

pol

A

LTR = initiates transcription, binds transcription factors

gag = encodes for the proteins inside the virus

env = encodes for the surface glycoproteins

pol: encodes the viral enzymes

50
Q

HIV utilizes a _______ molecule for a receptor and co-receptors may include _________, _________

A

CD4

Chemokine receptors –> CCR5, CXCR4

51
Q

Summarize the process of how HIV injects it’s HIV viral DNA into the host

A

HIV uses a CD4 molecule for a receptor to attach to the membrane, utilizes chemokine receptors to facilitate attachment.

Conformational change occurs

Gp41 acts as a “drill” and penetrates the membrane to facilitate membrane fusion to inject the HIV RNA genome into the host

52
Q

Describe what occurs once the HIV RNA genome is inside the host cell

A

Reverse transcriptase synthesizes proviral double stranded DNA from the original RNA genome

Integrase inserts the proviral DNA sequence into the host genome

Host cell activation occurs, triggering LTR to initiate transcription of HIV viral RNA

53
Q

NF-kB and HIV infection

Antigenic stimulation causes release of NF-kB

What is NF-kB supposed to do?

What does it actually do?

A

Supposed to: upregulate the T cell response

Actually does: Initiates viral transcription through the LTR

54
Q

What is responsible for host cell death in HIV infection?

A

The viral replication within the cell

(Direct cytopathic effect)

55
Q

What is pyroptosis?

A

Inflammasome-mediated programmed death pathway

It is responsible for cell death in non-replicating viral infections

56
Q

Although HIV LOVES CD4+ cells, it can also infect…

A

Macrophages

Dendritic Cells

Microglia

57
Q

B Lymphocytes in HIV infection

What is the general response?

A

Proliferative –> potentially due to secondary infection

Or may become clonal -lymphoma

58
Q

Describe the pathogenesis of HIV infection

A
59
Q

What are the major hallmarks in the timeline of HIV infection?

A

Day 7-14 = Viremia (viral load spikes)

Following day 16 = Seroconversion, (going from antibody (-) to antibody (+). 70% of people get symptoms

60
Q

Testing for HIV

What is the first detectable substance?

What test do you use for this?

A

Viral RNA

Nucleic Acid Test (NAT)

61
Q

Testing for HIV

What is the second detectable substance?

A

Protein antigen p24

62
Q

Testing for HIV

What is the third detectable substance?

A

Antibody to HIV

63
Q

What constitutes the diagnosis of AIDS?

A

Declining CD4+ counts and/or the onset of opportunistic infection or neoplasms

64
Q

What lab value constitues AIDS?

A

CD4+ T cell count that is

LESS THAN 200

65
Q

What diagnosis is likely with these findings?

A

CNS Toxoplasmosis

AIDS

66
Q

What is this an example of?

A

Opportunistic fungal infection that is an AIDS defining illness

(Pneumocystisi jiroveci)

67
Q

What is the major diarrhea inducing protozoa that Dr. Signh focused on?

A

Cryptosporidium

68
Q

What are these?

A

Kaposi Sarcomas

Vascular tumors that are a manifestation of AIDS

69
Q

AIDS related lymphomas

What is the primary mechanism to induce lymphomas?

A

HIV alters B-cell proliferation

OR

Reactivation of viruses (eg Epstein Barr Virus)

70
Q

Name a concerning AIDS defining illness

A

CANCER

(cervical/anal cancer)

71
Q

What co-infection is important in the development of Cancer driven by AIDS?

A

HPV

72
Q

Describe Amyloidosis

A

Protein synthesis and secretion is normally tightly regulated

However

In the cases of mutations, misfolding occurs resulting in insoluble beta-pleated sheets

(if it isn’t broken down, it can accumulate)

73
Q

(Amyloid)

What is AL?

A

Light chain diseases

74
Q

(Amyloid)

What is AA?

A

Amyloid associated

75
Q

Where does amyloid tend to manifest?

A

Kidney

Liver

Brain

Heart

76
Q

What is the special stain performed to identify amyloid?

A

Congo red stain

77
Q
A

MOD Exam II (6 decks)

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