T4 - L4 immunodeficiency Flashcards

(62 cards)

1
Q

why does the immune system need to carry out self-regulation?

A

important to minimise host damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

features of the innate immune system?

A
  • general/recognises broad classes e.g. bacteria
  • rapid onset
  • response doesn’t change with repeated exposure
  • no memory (same response each time)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

features of the adaptive immune system?

A
  • antigen specific
  • slower response
  • more potent response
  • memory
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what is immunodeficiency?

A

the immune system is not effective enough to protect the body against infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what is primary immunodeficiency?

A

Inherent defect within the immune system- usually genetic.

rare

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is secondary immunodeficiency?

A

Immune system affected due to external causes

[e.g. burns, massive cut]

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what are causes of secondary immunodeficiency?

A
  • break down of physical barriers
  • protein loss
  • malignancy
  • drugs
  • infection
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

how can protein loss cause secondary immunodeficiency?

A

d unable to produce the Ab needed

for the infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

how can lymphoma cause secondary immunodeficiency?

A
  • cancer of lymphocyte s
  • expands in the
    bone marrow, limiting the space for other immune system parts to develop
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

how can steroids cause secondary immunodeficiency?

A
  • Steroids work by supressing the immune system

- Recurrent courses of steroid can cause immunodeficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

how can DMARDS cause secondary immunodeficiency?

A

(Disease-modifying anti-rheumatic drugs)

  • Work by suppressing the immune system
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

how can Rituximab cause secondary immunodeficiency?

A
  • Rituximab works against B-cells
  • Clearing B-cells causes no antibody production
  • Excessive use could cause immunodeficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

how can Myelosuppressive cause secondary immunodeficiency?

A

Myelosuppressive works by suppressing the bone marrow where the
immune system is generated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

which drugs can cause secondary immunodeficiency?

A

Steroids, DMARDS, Rituximab, anti-convultants, myelosuppressive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Phagocytes including neutrophils and macrophages are capable of engulfing and absorbing bacteria. Which phagocyte out of the two is short-lived?

A

[Neutrophils are short lived (hours)]

[Macrophages are long lived (days, weeks)]

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what do lysosomes produce during phagocytosis in order to kill pathogens?

A

hypochlorous acid (i.e. bleach)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

how do pathogens know whether an antigen is self or foreign?

A
Pathogen recognition receptors (PRRs) - recognise conserved pathogen associated
molecular patterns (PAMPs) which are unique to each pathogen
  • phagocytes have PRRs for PAMPs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q
what is pathogen associated
molecular patterns (PAMPs)?
A

[PAMPs are things found in most or a group of bacteria, which differentiate them
from the human’s cells]

  • unique to each pathogen
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

give an examples of a common PAMPs?

A

Lipopolysaccharide

flagellin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what PRR recognises the PAMP Lipopolysaccharide?

A

Toll Like Receptor 4 (TLR 4)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what PRR recognises the PAMP flagellin?

A

TLR5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what PRR recognises the viral RNA?

A

TLR3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what happens when a phagocyte detects a pathogen via a PRR?

A

Cascade of events → production of inflammatory
cytokines

MyD88 and IRAK4 are involved in this cascade

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Once a phagocyte has recognised a PAMP via a PRR, what two proteins are involved in the following cascade?

A

MyD88 and IRAK4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
what happens in IRAK4 deficiency?
phagocytes can recognise the pathogen but cannot cause an inflammatory response
26
what is the clinical presentation of IRAK4 deficiency?
- Recurrent bacterial infection - Poor inflammatory response - Susceptibility to infection decreases with age
27
how would you treat IRAK4 deficiency?
prophylactic antibiotics, iv immunoglobulin if severe
28
what is a phagolysosome?
when a bug has been ingested by a phagocyte and is attached to a lysosome
29
what is a NADPH complex?
complex of proteins incl gp91phox (coded by the X chromosome) found on the surface of lysosomes
30
how does the NADPH complex help phagocytosis?
- releases an electron within the lysosome - binds to 02 forming superoxide - superoxide gives rise to hypocholorus acid - which kills pathogen
31
how is hpocholorus acid formed?
- NADPH complex - releases an electron within the lysosome - binds to 02 forming superoxide - superoxide gives rise to hypocholorus acid
32
what is a granuloma?
collection of macrophages
33
what causes chronic granulomatous disease?
- NADPH complex dysfunction - Mutation in gp91phox, which is encoded by X-chromosomes - X-linked condition
34
what happens in chronic granulomatous disease?
- NADPH complex dysfunction - Can ingest the bug but cannot destroy it - But still attracting neutrophils and macrophages in the area - Causing granuloma
35
what is the treatment for chronic granulomatos disease?
haemopoeitic stem cell transplant, antibiotics NB: [Bone marrow transplant is the standard treatment for Chronic Granulomatous Disease, replacing the entire immune system]
36
how do you diagnose Chronic Granulomatos Disease?
Measure Dihydrorhodamine reduction (gain of electron) OR Nitro blue tetrazolium dye reduction – healthy neutrophils should go [from clear to] purple - [Healthy individuals show change in neutrophil colour to purple] - [Patients with CGD show no change in neutrophil colour] - [Carriers of CGD show change in some of the neutrophils to purple]
37
what 3 things do complement proteins do?
- cell lysis (kill invading bacterium) - control of inflammation - stimulate phagocytosis
38
what 3 pathways stimulates the complement cascade?
classical pathway alternative pathway mannose-binding lectin (MBL) pathway
39
what is the end product of the complement cascade?
Membrane Attack Complex (MAC) - causes cell lysis and rupture (death)
40
how can complement deficiency lead to autoimmunity?
- Complement helps with clearing dead tissues | - If a person cannot clear them, they become prone to autoimmunity
41
what conditions can a C2, C4 complement deficiency lead to?
SLE infections myositis
42
what is myositis?
autoimmunity against muscles
43
what condition can a C5-C9 complement deficiency lead to?
bacterial meningitis C5-C9 (form membrane attack complex) - usual treatment: prophylactic penicillin B
44
repeated episodes of bacterial meningitis would indicate what type of autoimmune disease?
C5-C9 complement deficiency
45
how does an antibody inactivate an antigen when it binds?
- neutralisation - agglutination of microbes - precipitation of dissolved antigen NB: all activate complement cascade and leads to cell lysis
46
what is meant by neutralisation?
antibody blocks viral binding sites/coats bacteria
47
what is meant by agglutination?
antibodies stick microbes together
48
what is meant by precipitation of dissolved antigen?
stick antigens together
49
X linked agammaglobulinaemia is a genetic defect in what?
Bruton’s Tryrosine kinase - Needed for B cell signalling and B cell maturation - B cell maturation not completed - antibodies can not be produced
50
what conditions. can arise due to B cell defects?
- CVID - IgA deficiency - X Linked hyper IgM syndrome - Transient hypogammaglobulinaemia of infancy
51
what happens if there are defects in B cells?
- loss of antibody secretion - Usually leads to recurrent bacterial infection with pyogenic organisms - Treat with [prophylactic] antibiotics [e.g. azithromycin] then i.v IgG for life.
52
conditions that arise due to B cell defects are mostly very serious - however which one is asymptomatic?
IgA deficiency - [Common] - [Mostly asymptomatic but some people develop recurrent infections and become prone to autoimmune diseases]
53
how do you treat an antibody deficiency?
➢ Antibiotics | ➢ Immunoglobulin G replacement
54
what is Severe Combined Immunodeficiency (SCID)?
inherited abnormality in immune system - v high risk of infection
55
which would be more severe, B cell defects or T cell defects?
Defects in T cells Usually more severe since B cells also need T cell help
56
SCID is due to which type of cell defect?
T cell defect
57
no T cells would suggest which condition?
Severe Combined Immunodeficiency (SCID)
58
what causes SCID?
➢ Defect/absence of critical T cell molecule ➢ Loss of communication - MHCII deficiency o [B-cells not able to communicate with T-cells] ➢ Metabolic - Adenosine deaminase deficiency
59
how would a defect in a phagocyte present?
PRR: IRAK4→ recurrent pneumonia, poor inflammatory response
60
how would defects in the complement cascade present?
bacterial meningitis
61
how would defects in antibodies present?
recurrent infection
62
how would defects in T-cells present?
SCID