Immunocompromise Flashcards

1
Q

Define ‘immunity’.

A

Ability of a host (usually your patient) to resist invasion and damage by pathogens (usually micro-organisms such as bacteria, viruses, fungi or parasites).

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2
Q

Define immunocompetence.

A

‘Normal’ immunity, conferring similar ability to resist invasion and damage by pathogens as other indiviuals in the population.

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3
Q

Define immunocompromise.

A

Attenuated or absent immuity, leaving the patient more vulnerable to invasion and damage by pathogens than others in the population.

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4
Q

What causes the innate immune system to be compromised?

A
  • Broken skin
    • Cellulitis and abscesses
  • Base of skull fractures
    • ​Pneumococcal meningitis
  • Ciliary dysfunction
    • Increased respiratory infection
  • Bladder outflow obstruction
    • UTI and pyelonephritis
  • Chemotherapy
    • Bone marrow suppression and neutropaenia
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5
Q

What causes the adaptive immune system to be compromised?

A
  • HIV
    • Immune cell depletion (especially helper (CD4) T cells)
  • Drugs (e.g. for autoimmune disease / post-transplant)
    • Deliberate targeted suppression of specific immune activities
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6
Q

What are the 3 primary organs of the immune system and what causes them to become compromised?

A
  • Thymus
    • Key organ of T-cell development, especially in children.
    • Failure to develop or dysfunction causes T cell deficiency.
  • Bone marrow
    • Site of WBC synthesis.
    • Dysfunction (e.g. leukaemia, drugs) causes neutropaenia ad other cell subset deficiencies.
  • Spleen
    • Synthesises antibodies, removes antibody-coated bacteria, stores monocytes and lymphocytes.
    • Loss increases the risk of sepsis from encapsulated bacteria.
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7
Q

Describe primary (congenital) immune deficiency.

A
  • Rare
  • Often hereditary
    • (Autosomal recessive or X-linked)
  • Some causes:
    • Di George’s syndrome
    • X-linked agammaglobulinaemia
    • Severe combined immunodeficiency
    • Ataxia telangiectasia
    • Chronic granulomatous disease
    • Complement deficiencies
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8
Q

Describe secondary (acquired) immune deficiency.

A
  • Common
  • Multiple causes
  • Risk factors:
    • Extremes of age
    • Concurrent illness
      • Cancer
      • DM
      • Organ failure (e.g. liver cirrhosis)
  • Some causes:
    • HIV
    • Splenectomy (trauma, infarct, surgery)
    • Immune suppressive medication
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9
Q

What is the relationship between immune deficiency and immune dysregulation?

A
  • Immune deficiency
    • Increased predisposition to infection.

OFTEN GOES TOGETHER WITH

  • Immune dysregulation
    • Inappropriate inflammatory responses.
    • Adverse drug reactions.
    • Higher risk of malignancy.
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10
Q

Describe the charateristics of infection in immunocompromised patients.

A
  • Often chronic, severe or recurrent.
  • May only partially resolve after ABx therapy.
  • The organisms involved are often unusual (‘opporunistic’).
  • Clinical presentation can by atypical.
  • Risk of antimicrobial resistance.
  • The organisms involved are influenced by the nature of the immune defect.
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11
Q

What are the possible causes and high risk pathogens for neutropaenia or neutrophil dysfunction?

A
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12
Q

What are the possible causes and high risk pathogens for complement deficiency?

A
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13
Q

What are the possible causes and high risk pathogens for T cell deficiency?

A
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14
Q

What are the possible causes and high risk pathogens for B cell / antibody deficiency?

A
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15
Q

What are the warning signs for primary immunodeficiency?

A
  • Sometimes part of multi-system ‘syndromes’ with other key features.
  • Often identified in early childhood. Consider if:
    • Recurrent ear / sinus / chest infections
    • Failure to gain weight or meet growth milestones
    • Persistent oral candida in mouth, or fungal infections on skin
    • ≥2 deep-seated infections, including sepsis
    • FHx of primary immunodeficiency
  • Investigation and management requires speciality immunology input.
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16
Q

Describe Di George syndrome.

A
  • 1-5 / 100,000 live births.
  • Autosomal dominant deletion on chromosome 22.
  • Failure of 3rd & 4th pharyngeal arches to develop.
    • Parathyroid gland, aortic arch and thymus do not develop.
    • Dysmorphic faces, hypo-PTH.
  • Increased incidence of fungal and protozoan infection.
  • Specialist care includes:
    • Calcium supplementation
    • Correction of cardiac abnormalities
    • Prophylactic ABx
    • Thymic transplantation
17
Q

Give examples of other conditions which cause primary T & B lymphocyte deficiencies.

A
  • Severe combined immunodeficiencies (SCID)
  • Hyper IgM syndrome
  • Ataxia telangiectasia
18
Q

Give examples of conditions which cause primary B lymphocyte deficiency.

A
  • X-linked agammaglobulinaemia (XLA)
  • Selective IgA deficiency
  • Common variable immunodeficiency (CVID)
19
Q

Give examples of conditions which cause primary neutrophil function deficiency.

A
  • Chronic granulomatous disease
  • Leucocyte adhesion defect (LAD)
20
Q

What is neutropaenia?

Define mild, moderate and severe.

A
  • A decrease in peripheral blood neutrophil count.
  • Mild - 1.0-1.5 x 109 /L
  • Moderate - 0.5-1.0 x 109 /L
  • <0.5 x 109 /L
  • Common in oncology patients post-chemotherapy (day 8-10 nadir), post-radiotherapy, or due to marrow infiltration by malignancy.
21
Q

What is febrile neutropaenia?

A
  • Neutropaenia (especially severe) and temperature >38.
  • 50-60% of febrile neutropaenic patients will have infection.
  • 16-20% of febrile neutropaenic patients will have bacteraemia.
  • Sepsis may present without a focal source.
  • The illness may progress rapidly.
22
Q

Describe the treatment and prevention of neutropaenia and sepsis.

A
  • Look carefully for an infective source including lines.
  • Treatment:
    • Remember your sepsis 6
    • IV ABx within the first hour
      • Piperacillin-tazobactam + gentamicin or meropenem
  • Prevention:
    • Vaccines up to date?
    • Screen for opportunistic infections
    • Prophylactic medications
    • IVIg and G-CSF
23
Q

Which drugs can be implicated in immunocompromise?

A
  • Corticosteroids
  • Disease-modifying antirheumatic drugs (DMARDs)
    • Conventional
      • Azathioprine
      • Hydroxychloroquine
      • Methotraxate
      • Sulfasalazine
    • Biological
      • Abatacept
      • Adalimumab
      • Etanercept
      • Infliximab
      • Rituximab
      • Tocilizumab
    • Organ transplantation and anti-rejection medication
      • Cyclosporin
      • Tacrolimus
24
Q

17-year old trauma patient.

Spleen removed after road traffic accident.

What are the specific risks? What should be done?

A
  • Highest risk is sepsis from capsulated organisms.
  • Overwhelming post-splenectomy infection (OPSI)
    • ~0.5% per year, 5% lifetime risk
  • Vaccinations:
    • Pneumococcal
    • H. influenzae b
    • Meningococcal influenzae
  • Preventative ABx:
    • At least 2 years, maybe lifelong
25
Q

What are the contibutory factors to immune compromise in patients with chronic respiratory disease?

What steps can be taken in infection prevention?

A
  • Cigarette smoking
  • Ciliary impairment
  • Altered innate and adaptive immunity
  • Recurrent corticosteroids and ABx
  • Infection prevention
    • Stop smoking
    • Judicious use of medication
    • Influenzae and pneumococcal vaccination
26
Q

How does diabetes mellitus affect immunosuppression?

How can infection be prevented in this group?

A
  • Increased risk of UTIs
  • Increased skin, soft tissue, bone and joint infection
  • Evidence of association with TB
  • Infection prevention:
    • Try to optimise glycaemic control
    • Meticulous foot care
    • Influenza and pneumococcal vaccination
27
Q

Chronic heart / liver / kidney disease can cause individuals to be immunocompromised.

How can infection be prevented in this group?

A
  • Influenza and pneumococcal vaccination
  • Also Hep A and B vaccination in liver cirrhosis