Immunology

Question 1

What is immunodeficiency

Answer 1

chronic infection
defective immune response
pathogens aren’t cleared

Question 2

What are clinical signs of immune deficiency

Answer 2

serious infections
persistent infections
unusual infections
recurrent infections

Question 3

what makes an infection ‘serious’

Answer 3

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

Question 4

what are other features someone with immunodeficiency would display?

Answer 4

weight loss
failure to thrive
diarrhoea
cancer

Question 5

what is a primary immunodeficiency disorder

Answer 5

genetic
at birth
not acquired from elsewhere

Question 6

what are secondary immunodeficiency disorders

Answer 6

acquired from something
usually affects more than 1 cell type

Question 7

what infections are associated with secondary immune deficiency

Answer 7

Human immunodeficiency virus
measles

Question 8

What treatment interventions are associated with secondary immune deficiency

Answer 8

immunosuppressive drugs
anti-cancer agents
corticosteroids

Question 9

What biochemical/nutritional disorders are associated with secondary immune deficiency

Answer 9

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

Question 10

what are the classifications of primary immunodeficiency disorders

Answer 10

immune dysregulation
auto inflammatory disorders
defects in innate or adaptive immunity

Question 11

What PID results in the earliest manifestation and mortality

Answer 11

respiratory diseases

Question 12

What are upper respiratory complications caused by PID

Answer 12

sinusitis
otitis media
laryngeal angioedema

Question 13

what are the lower respiratory complications associated with respiratory PID

Answer 13

malignancies
interstitial lung disease
pneumonia
bronchitis

Question 14

Where can defects occur in neutrophils leading to immune disorders

Answer 14

development
trans-endothelial migration
killing by neutrophils

Question 15

what defects in neutrophil development can occur

Answer 15

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

Question 16

How can defects in neutrophil development be treated

Answer 16

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

Question 17

How can defects in neutrophil trans-endothelial migration occur

Answer 17

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

Question 18

What are the clinical features in patients with neutrophils lacking ICAM1

Answer 18

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

Question 19

What defects in neutrophil killing can occur

Answer 19

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

Question 20

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

Answer 20

redcurrant fungal infections
recurrent bacterial infections
granuloma formation

Question 21

Why do granulomas form when neutrophils lack the NADPH oxidase complex

Answer 21

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

Question 22

What are the main treatments for phagocyte deficiencies

Answer 22

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

Question 23

What is SCID

Answer 23

Severe combined immunodeficiency

Question 24

what are the causes of SCID

Answer 24

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

Question 25

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

Answer 25

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

Question 26

What is IL-2

Answer 26

T cell growth factor

Question 27

What are the clinical phenotypes of SCID patients

Answer 27

unwell in early childhood diarrhoea failure to thrive infections skin disease

Question 28

Why do SCID patients have recurrent viral infections

Answer 28

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

Question 29

Why are bacterial infections recurrent in SCID patients

Answer 29

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

Question 30

why are fungal infections frequent in SCID patients

Answer 30

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

Question 31

What are the prophylactic treatments for SCID patients

Answer 31

prophylactic antibiotics prophylactic antifungals no vaccines replacement immunoglobulin

Question 32

What is the definitive treatment for SCID

Answer 32

stem cell transplant from MHC class identical donor

Question 33

Which gene is essential for B cell development

Answer 33

BTK

Question 34

What are the disorders of T cell effector function

Answer 34

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

Question 35

When are long-lived memory T and B cells produced

Answer 35

Primary immune response

Question 36

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

Answer 36

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