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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
What are the clinical manifestations of **DiGeorge syndrome?**
- Facial and palatal abnormalities - Cardiac abnormalities - Tetany - Immune deficiency
26
X-Linked Agammaglobulinemia Aka? Mechanism?
"Bruton's agammaglobulinemia" Inability of **pre-B cells to mature**
27
Generally, what is the presentation of **agammaglobulinemia?**
Encapsulated bacteria
28
Hyper-IgM syndrome What kind of mutation? What does this cause? How do you treat?
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**
29
Common Variable Immunodeficiency (CVID) A collection of disorders resulting in...
Hypogammaglobulinemia
30
What are some manifestations of **Common Variable Immunodeficiency (CVID)?**
Recurrent infections Granulomas Chronic diarrhea (Giardia) Autoimmune disease
31
IgA Deficiency (isolated) Describe:
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
What is potentially a first indication a patient has an **IgA deficiency?**
Transfusion-related anaphylaxis
33
Wiskott Aldrich syndrome Gene mutation?
WASP gene mutation
34
Wiskott Aldrich syndrome Symptoms?
Triad of: 1. Thrombocytopenia 2. Eczema 3. Recurrent infections
35
Wiskott Aldrich Syndrome Treatment?
Stem cell transplant
36
Ataxia Telangiectasia What kind of disease? What is a common sign? Major problems that arise? Inheritance pattern?
Neurodegenerative disease Vascular malformation (can see in sclera of eyes) Immune deficiency causes, **respiratory infections, autoimmune disease, cancer** **AR**
37
What can cause **secondary immunodeficiency?**
Cancer Malnutrition Metabolic disease Chronic illness Treatment-related (chemotherapy, radiation, immunosuppression) Acquired immunodeficiency syndrome (AIDS)
38
# Define: ## Footnote **Aquired Immunodeficiency syndrome (AIDS)**
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
How does HIV spread?
Sex w/out a condom Passed from mother to baby Sharing injecting equipment Contaminated blood transfusions and organ transplants
40
Who is at risk for aquiring HIV?
- 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
HIV Transmission via transfusions What are some important things to note with this mode of transmission?
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
HIV transmission from mother to child What are the ways this can occur?
**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
What is the likelihood that you will get infected with.... HIV Hepatitis C following a needle stick accident
HIV = 0.3% Hepatitis C = 30%
44
What is the classification of **HIV?**
Retrovirus Lentivirus family
45
HIV structure and Content What is an important **capsid protein?** Why do we care?
p24 We can test for p24 antigen
46
HIV structure and Content What are important **glycoproteins**? Why do we care?
**gp120** and **gp41** Important for attachment GOOD DRUG/VACCINE TARGETS
47
HIV structure and Content What are the important **viral enzymes**?
Protease Reverse transcriptase Integrase
48
There are 4 high yield regions Dr. Singh wants us to know about in the HIV-1 genome. What are they?
LTR gag env pol
49
What is the function of each region listed below in HIV-1? LTR gag env pol
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
HIV utilizes a _______ molecule for a receptor and co-receptors may include \_\_\_\_\_\_\_\_\_, \_\_\_\_\_\_\_\_\_
CD4 Chemokine receptors --\> CCR5, CXCR4
51
Summarize the process of how HIV injects it's HIV viral DNA into the host
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
Describe what occurs once the HIV RNA genome is **inside the host cell**
**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
NF-kB and HIV infection Antigenic stimulation causes release of NF-kB What is NF-kB supposed to do? What does it actually do?
Supposed to: upregulate the T cell response Actually does: Initiates **viral transcription** through the **LTR**
54
What is responsible for host cell death in HIV infection?
The viral replication within the cell (Direct cytopathic effect)
55
What is **pyroptosis?**
Inflammasome-mediated programmed death pathway It is responsible for cell death in non-replicating viral infections
56
Although HIV LOVES CD4+ cells, it can also infect...
Macrophages Dendritic Cells Microglia
57
B Lymphocytes in HIV infection What is the general response?
**Proliferative** --\> potentially due to secondary infection Or may become **clonal -lymphoma**
58
Describe the pathogenesis of HIV infection
59
What are the major hallmarks in the timeline of HIV infection?
Day 7-14 = **Viremia** (viral load spikes) Following day 16 = **Seroconversion**, (going from antibody (-) to antibody (+). 70% of people get symptoms
60
Testing for HIV What is the **first detectable substance?** What test do you use for this?
Viral RNA Nucleic Acid Test (NAT)
61
Testing for HIV What is the second detectable substance?
Protein antigen p24
62
Testing for HIV What is the third detectable substance?
Antibody to HIV
63
What constitutes the diagnosis of AIDS?
Declining **CD4+** counts and/or the **onset of opportunistic infection** or **neoplasms**
64
What lab value constitues AIDS?
CD4+ T cell count that is LESS THAN 200
65
What diagnosis is likely with these findings?
CNS Toxoplasmosis AIDS
66
What is this an example of?
Opportunistic fungal infection that is an **AIDS defining illness** (Pneumocystisi jiroveci)
67
What is the major diarrhea inducing protozoa that Dr. Signh focused on?
Cryptosporidium
68
What are these?
**_Kaposi Sarcomas_** Vascular tumors that are a manifestation of AIDS
69
AIDS related lymphomas What is the primary mechanism to induce lymphomas?
HIV alters **B-cell proliferation** OR Reactivation of viruses (eg Epstein Barr Virus)
70
Name a concerning **AIDS defining illness**
CANCER (cervical/anal cancer)
71
What co-infection is important in the development of **Cancer driven by AIDS?**
HPV
72
Describe **Amyloidosis**
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
(Amyloid) What is **AL?**
Light chain diseases
74
(Amyloid) What is AA?
Amyloid associated
75
Where does amyloid tend to manifest?
Kidney Liver Brain Heart
76
What is the special stain performed to identify **amyloid?**
Congo red stain
77

MOD Exam II (6 decks)

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