Immunology: Primary Immune Deficiencies Flashcards

1
Q

Primary Immune Deficiencies:

What are the CD4, CD8, B Cell, IgM and IgG changes in:

  1. SCID
  2. Di George
  3. BLS
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Primary Immune Deficiencies:

What are the CD4, CD8, B Cell, IgM, IgG and IgA changes in:

  1. SCID
  2. Brutons
  3. Hyper IgM
  4. Selective IgA Deficiency
  5. CVID
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Primary Immune Deficiencies:

What are the Neutrophil count, Leukocyte adhesion markers, Nitroblue test of oxidative killing (NBT) and pus findings in:

  1. Kostmann syndrome
  2. Leukocyte adhesion deficiency
  3. Chronic Granulomatous disease
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Primary Immune Deficiencies:

What is are the subclasses of Primary immune deficiencies?

A

“BACKLIT CHAMP R”

  • B Lymphocyte Maturation
  • Alternative pathway
  • Classical pathway
  • Killing
  • Lymphoid progenitors
  • Immune barriers
  • Terminal common pathway/T Cell maturation/T Cell acitvation
  • C3
  • HAem stem cells
  • Mannose binding lectin/Mobilisation of Phagocytes
  • Phagocytosis
  • Recruitment of other cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Primary Immune Deficiencies:

What are the features, disease and disease characteristics of External epithelia deficiencies?

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

Primary Immune Deficiencies:

What are the features, disease and disease characteristics of Mucosal surfaces deficiencies?

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

Primary Immune Deficiencies:

What are the features, disease and disease characteristics of Commensal bacteria deficiencies?

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Reticular dysgenesis?

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

Primary Immune Deficiencies:

What are the features and disease characteristics of Kostmann syndrome?

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Cyclic neutropenia?

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

Primary Immune Deficiencies:

What are the features and disease characteristics of Leukocyte adhesion deficiency?

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Primary Immune Deficiencies:

What are the features, disease and disease characteristics of Phagocytosis deficiencies?

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Chronic granulomatous disease? Including:

  1. Absent respiratory burst
  2. Excessive inflammation
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Chronic granulomatous disease? Including:

  1. Symptoms
  2. Investigations performed
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Primary Immune Deficiencies:

What are the features, disease and disease characteristics of Recruitment of other cell deficiencies?

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

Primary Immune Deficiencies:

What are the features, disease and disease characteristics of Alternative Pathway deficiencies?

A
17
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Deficiencies in early classical Pathway (C1/2/4)?

Include:

  1. Aetiolgy
  2. Depoisition sites
A
18
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Deficiencies in early classical Pathway (C1/2/4)?

Include:

  1. Complement deficiency
  2. Clinical phenotype
A
19
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Secondary Classical Pathway deficiencies?

A
20
Q

Primary Immune Deficiencies:

What are the features, disease and disease characteristics of Mannose binding lectin deficiencies?

A
21
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of C3 Deficiencies?

A
22
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Secondary C3 deficiencies?

A

Don’t confuse with secondary Classical pathway deficiencies, which are caused by active lupus depleting the complement

23
Q

Primary Immune Deficiencies:

What are the features, disease and disease characteristics of terminal common pathway deficiencies?

A
24
Q

Primary Immune Deficiencies:

What are the features, disease and disease characteristics of Haem stem cell deficiencies?

A
25
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of SCID?

Include:

  1. Clinical presentation
  2. Family history
A
26
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of SCID?

Include:

  1. Associated Pathways
  2. Effect on Lymphocytes
A
27
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of X-linked SCID? Include:

  1. Epidemiology
  2. Genetics
A
28
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of X-linked SCID? Include:

  1. Phenotype
A
29
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of DiGeorge syndrome?

A
30
Q

Primary Immune Deficiencies:

What is the clinical presentation of Bare lymphocyte Type II syndrome?

A
31
Q

Primary Immune Deficiencies:

What is the aetiology of Bare lymphocyte Type II syndrome?

A
32
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Bruton’s X-linked hypogammaglobulinemia?

A
33
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Hyper IgM Syndrome? Include:

  1. Definition
  2. Genetics
A
34
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Hyper IgM Syndrome? Include:

  1. Symptoms
  2. Complications
A
35
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Common variable immune deficiency?

A
36
Q

Primary Immune Deficiencies:

What is are the subclasses of Primary immune deficiencies affecting Immune barriers?

A

Immune barriers:

  • External epithelia
  • Mucosal surfaces
  • Commmensal Bacteria
37
Q

Primary Immune Deficiencies:

What is are the subclasses of Primary immune deficiencies affecting:

  • Mobilisation of Pagocytes
  • T Cell activation and effector functions
  • T Cell maturation
  • B Lymphocyte Maturation
A

Mobilisation of Phagocytes:

  • Production of neutrophils
  • Neutrophil maturation
  • Migration to site of infection

T Cell activation and effector functions:

  • Cytokine release
  • T/B Cell communication

T Cell Maturation:

  • ​Thymus
  • Positive and negative selection
  • Exposure of mature T cells to periphery

B Lymphocyte Maturation:

  • ​Pro B Cells
  • Class switching