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CSIM 1: Immunology Strand > CSIM 1.30 Immunodeficiency > Flashcards

CSIM 1.30 Immunodeficiency Flashcards

1
Q

What are the three main functions of antibody?

A
  • Neutralisation
    • Opsonisation
    • Complement activation
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2
Q

What are the three main functions of complement?

A
  • Opsonisation
    • Clearance of immune complexes
    • Direct lysis of bacteria
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3
Q

What are the roles of neutrophils?

A
  • Phagocytosis of extracellular pathogens

* Activation of bacteriocidal mechanisms

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4
Q

What is Hib?

A

Haemophilus influenza type B

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5
Q

What are the congenital antibody deficiencies affecting IgG?

A
  • Transient hypogammaglobulinaemia of infancy
    • X-linked agammaglobulinaemia
    • AR agammaglobulinaemia
    • Common variable immunodeficiency
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6
Q

What is the half-life of IgG?

A

3 weeks

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7
Q

Why are congenital antibody deficiencies not apparent in the first few months of life? What is the normal onset time for congenital antibody deficiencies affecting IgG?

A

Because maternal IgG is transferred through the placenta

6-12 months

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8
Q

How is transient hypogammaglobulinaemia of infancy identified?

A
  • 6 months of age
    • Frequent upper respiratory tract infections
    • Low IgG levels as maternal IgG is depleted
    • Resolves eventually, once the infant’s own IgG begins to develop properly
    • CD19 is present (B cells)
    • IgM is being made (the problem is simply in switching to IgG)
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9
Q

What pathogens are commonly seen in humoral deficiencies?

A
  • S. aureus
    • S. pneumniae
    • H. influenzae
    • Enteroviruses
    • Echoviruses
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10
Q

Which congenital immunodeficiency defects affect phagocytes?

A

Neutropenia
• Defects in phagocyte production

Leukocyte adhesion deficiency I-III
• Defects in adhesion molecules affecting chemotaxis

Chronic granulomatous disease (XL and AR)
• Defects in killing mechanisms

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11
Q

How do those with chronic granulomatous disease present?

A
  • Liver abscess
    • Suppurative lymphadenitis (inflammed and infected lymph nodes)
    • Pulmonary infiltrates
    • Burkholderii septicaemia
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12
Q

Describe what happens in chronic granulomatous disease (CGD)

A

Phagocytes are able to phagocytose pathogens, but are unable to produce the chemicals needed to break them down (they have a defective oxidative burst)

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13
Q

How is CGD identified histologically?

What is seen in carriers?

A

Nitro-blue tetrazolium (NBT) dye test is used
• Dye changes to dark blue when exposed to superoxide

Carriers will have two populations of phagocytes: those with and without superoxide components (IMG 77)

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14
Q

What are the most common recurrent infections found in those with congenital complement deficiency and why?

A

Streptococci
Hib

Because opsonisation is critical for the removal of these pathogens

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15
Q

Deficiency in which complement proteins leads to recurrent meningococcal infection?

Which drug can therefore increase the risk of meningococcal infection?

A

C5-C9

Eculizumab (C5 inhibitor)

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16
Q

How is antibody, complement and neutrophil deficiency treated?

A
  • Antibiotic prophylaxis
    • Antifungal prophylaxis
    • Replacement antibodies
17
Q

What causes secondary B cell deficiencies?

A

Iatrogenic
• Rituximab
• Bortizumib
• Plasmapheresis

Pathogenic
• Nephrotic syndrome (loss of proteins such as immunoglobulins through glomerulus)

18
Q

What causes secondary phagocyte deficiencies?

A

• Eculizumab (C5 inhibitor)

19
Q

Why do T cell deficiencies usually coincide with B cell deficiencies?

What is the resulting condition called?

A

Because T cells activate B cells

Severe combined immunodeficiency (SCID)

20
Q

Describe SCID

A
  • Hospitalisation occurs 50-100 days
    • Fatal by 1 year of age
    • Low CD3, CD4, CD8 (and CD19)
    • Low Igs
    • Absolute lymphocyte count is
21
Q

How is T cell deficiency (SCID) treated?

A
  • Antibiotic prophylaxis
    • Antifungal prophylaxis
    • Replacement antibodies
    • Haemopoeitic stem cell transplantation
    • Gene therapy
22
Q

Define immunodeficiency

A

A failure to achieve immune function to provide efficient, self limited host defence against the biotic and abiotic environment while preserving tolerance to self

23
Q

What is IPEX syndrome?

A

Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked:
• Failure of peripheral self-tolerance
• Due to absence of Treg cells
• This is caused by mutations in FOXP3

24
Q

What is AIRE deficiency

A

Autoimmune receptor deficiency
• AIRE is usually responsible for expressing self-antigen in thymic cells
• This means that T cells recognising self antigen do not get deleted in the thymus

25
Q

What causes autoimmune lymphoproliferate syndrome?

A

• Loss of apoptosis of immune cells due to loss of Fas (death receptor) and FasL (death ligand) leading to loss of regulation of the immune system

Presentations:
• Liver failure and bone marrow failure caused by macrophages (autoimmunity)
• Haemophagocytic lymphohistiocytosis
• Ranging temperature

26
Q

Why does prematurity cause immunodeficiency?

A

Because the baby misses out on maternal IgG

27
Q

Which viral infections can cause secondary immunodeficiency?

A
  • HIV
    • Measles
    • Viral URTI

CSIM 1: Immunology Strand (17 decks)

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