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FoM > Immunology > Flashcards

Immunology Flashcards

1
Q

What is immunodeficiency

A

chronic infection
defective immune response
pathogens aren’t cleared

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2
Q

What are clinical signs of immune deficiency

A

serious infections
persistent infections
unusual infections
recurrent infections

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3
Q

what makes an infection ‘serious’

A

doesn’t respond to oral antibiotics (must be IV)

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4
Q

what are other features someone with immunodeficiency would display?

A

weight loss
failure to thrive
diarrhoea
cancer

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5
Q

what is a primary immunodeficiency disorder

A

genetic
at birth
not acquired from elsewhere

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6
Q

what are secondary immunodeficiency disorders

A

acquired from something
usually affects more than 1 cell type

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7
Q

what infections are associated with secondary immune deficiency

A

Human immunodeficiency virus
measles

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8
Q

What treatment interventions are associated with secondary immune deficiency

A

immunosuppressive drugs
anti-cancer agents
corticosteroids

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9
Q

What biochemical/nutritional disorders are associated with secondary immune deficiency

A

malnutrition (need nutrients to make WBC)
type 1 and 2 diabetes
mineral deficiencies

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10
Q

what are the classifications of primary immunodeficiency disorders

A

immune dysregulation
auto inflammatory disorders
defects in innate or adaptive immunity

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11
Q

What PID results in the earliest manifestation and mortality

A

respiratory diseases

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12
Q

What are upper respiratory complications caused by PID

A

sinusitis
otitis media
laryngeal angioedema

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13
Q

what are the lower respiratory complications associated with respiratory PID

A

malignancies
interstitial lung disease
pneumonia
bronchitis

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14
Q

Where can defects occur in neutrophils leading to immune disorders

A

development
trans-endothelial migration
killing by neutrophils

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15
Q

what defects in neutrophil development can occur

A

mutation in ELANE
enzyme needed for maturation
build up of precursor in bone marrow

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16
Q

How can defects in neutrophil development be treated

A

give individual recombinant cytokine that stimulates production of enzyme needed for maturation

17
Q

How can defects in neutrophil trans-endothelial migration occur

A

ICAM1 not expressed on neutrophils
leukocytes can’t stick to endothelial cells and move into tissues

18
Q

What are the clinical features in patients with neutrophils lacking ICAM1

A

redcurrant bacterial and fungal infections
high count of phagocytes in blood stream

19
Q

What defects in neutrophil killing can occur

A

mutation in gene coding for NADPH oxidase complex
therefore neutrophil cannot produce ROS
can still kill

20
Q

What are the clinical features in individuals with neutrophils lacking gene for NADPH complex

A

redcurrant fungal infections
recurrent bacterial infections
granuloma formation

21
Q

Why do granulomas form when neutrophils lack the NADPH oxidase complex

A

macrophage can’t kill bacteria so it surrounds it to try and starve it of nutrients

22
Q

What are the main treatments for phagocyte deficiencies

A

immunoglobin replacement therapy
management of infections through antibiotics
definitive therapies - stem cell transplant

23
Q

What is SCID

A

Severe combined immunodeficiency

24
Q

what are the causes of SCID

A

deficiency of cytokine receptors
deficiency of signalling molecules
metabolic defects
defective receptor arrangements

25
Q

What occurs if there is a mutation of a component of IL-2 receptor

A

inability to respond to cytokines
failure of T cell and NK development
produces immature B cells

26
Q

What is IL-2

A

T cell growth factor

27
Q

What are the clinical phenotypes of SCID patients

A

unwell in early childhood
diarrhoea
failure to thrive
infections
skin disease

28
Q

Why do SCID patients have recurrent viral infections

A

no CD8+T cells
therefore no cytotoxic T cells
Cells can’t recognise virally infected cells

29
Q

Why are bacterial infections recurrent in SCID patients

A

No Tfh cells which are needed to help B cells make antibodies

30
Q

why are fungal infections frequent in SCID patients

A

effector CD4+Tfh cells and antibodies are needed to fight fungal infections

31
Q

What are the prophylactic treatments for SCID patients

A

prophylactic antibiotics
prophylactic antifungals
no vaccines
replacement immunoglobulin

32
Q

What is the definitive treatment for SCID

A

stem cell transplant from MHC class identical donor

33
Q

Which gene is essential for B cell development

A

BTK

34
Q

What are the disorders of T cell effector function

A

cytokine production
cytotoxicity
T-B cell communication
killing of infected host cells

35
Q

When are long-lived memory T and B cells produced

A

Primary immune response

36
Q

Why do memory T and B cells have a more effective response (secondary)

A

present in greater numbers
persist in absence of antigens
less reliant on costimulatory molecules
already class switched

FoM (38 decks)

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