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
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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
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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
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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
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5
Q

Primary Immune Deficiencies:

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

A
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6
Q

Primary Immune Deficiencies:

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

A
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7
Q

Primary Immune Deficiencies:

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

A
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8
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Reticular dysgenesis?

A
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9
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Kostmann syndrome?

A
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10
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of Cyclic neutropenia?

A
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11
Q

Primary Immune Deficiencies:

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

A
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12
Q

Primary Immune Deficiencies:

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

A
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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
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14
Q

Primary Immune Deficiencies:

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

  1. Symptoms
  2. Investigations performed
A
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15
Q

Primary Immune Deficiencies:

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

A
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16
Q

Primary Immune Deficiencies:

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

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
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
19
Q

Primary Immune Deficiencies:

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

20
Q

Primary Immune Deficiencies:

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

21
Q

Primary Immune Deficiencies:

What are the features and disease characteristics of C3 Deficiencies?

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?

24
Q

Primary Immune Deficiencies:

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

25
**_Primary Immune Deficiencies:_** What are the features and disease characteristics of SCID? Include: 1. Clinical presentation 2. Family history
26
**_Primary Immune Deficiencies:_** What are the features and disease characteristics of SCID? Include: 1. Associated Pathways 2. Effect on Lymphocytes
27
**_Primary Immune Deficiencies:_** What are the features and disease characteristics of X-linked SCID? Include: 1. Epidemiology 2. Genetics
28
**_Primary Immune Deficiencies:_** What are the features and disease characteristics of X-linked SCID? Include: 1. Phenotype
29
**_Primary Immune Deficiencies:_** What are the features and disease characteristics of DiGeorge syndrome?
30
**_Primary Immune Deficiencies:_** What is the clinical presentation of Bare lymphocyte Type II syndrome?
31
**_Primary Immune Deficiencies:_** What is the aetiology of Bare lymphocyte Type II syndrome?
32
**_Primary Immune Deficiencies:_** What are the features and disease characteristics of Bruton’s X-linked hypogammaglobulinemia?
33
**_Primary Immune Deficiencies:_** What are the features and disease characteristics of Hyper IgM Syndrome? Include: 1. Definition 2. Genetics
34
**_Primary Immune Deficiencies:_** What are the features and disease characteristics of Hyper IgM Syndrome? Include: 1. Symptoms 2. Complications
35
**_Primary Immune Deficiencies:_** What are the features and disease characteristics of Common variable immune deficiency?
36
**_Primary Immune Deficiencies:_** What is are the subclasses of Primary immune deficiencies affecting Immune barriers?
**_Immune barriers:_** * External epithelia * Mucosal surfaces * Commmensal Bacteria
37
**_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
**_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