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[ ESA 3- Infection and Immunity > The Immunocompromised Host > Flashcards

The Immunocompromised Host Flashcards

1
Q

What is meant by immunocompromisation?

A

State in which the immune system is unable to respond appropriately and effectively in infectious microorganisms

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2
Q

What is immnocompromisation due to?

A

A defect in one or more components of the immune system

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3
Q

What can infections suggest?

A

An underlying immune deficiency

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4
Q

When can infections suggest an underlying immune deficiency?

A

If they meet the criteria of SPUR

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5
Q

What is in the SPUR criteria?

A

Severe
Persistent
Unusual
Recurrent

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6
Q

What is considered to be a severe infection?

A

Life threatening

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7
Q

What is considered to be a persistent infection?

A

Minimal response to standard treatment

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8
Q

What factors may be considered unusual in an infection?

A

Site

Type of microbe

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9
Q

What site might be considered unusual in an infection?

A

Deep tissue

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10
Q

What type of microbe may be considered unusual in an infection?

A

Opportunistic infection

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11
Q

What is meant by a recurrent infection?

A

Returns regularly after treatment

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12
Q

What are the types of immunodeficiency?

A

Primary

Secondary

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13
Q

What kind of conditions are primary immunodeficiencies?

A

Congenital

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14
Q

What are primary immune deficiencies due to?

A

Intrinsic cell defect

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15
Q

How many intrinsic cell defects are there causing primary immunodeficiencies?

A

~275 genes

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16
Q

What can intrinsic cell defects lead to in primary immune deficiencies?

A

Missing protein
Missing cell
Non-functional components

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17
Q

What kind of conditions are secondary immunodeficiencies?

A

Acquired

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18
Q

What are secondary immune deficiencies due to?

A

Underlying disease/treatment leading to ;
Decreased production/function of immune components
Increased loss or catabolism of immune components

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19
Q

Do primary immune deficiencies affect the innate or adaptive immune system?

A

Can be either

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20
Q

What conditions have a decreased production of immune components as a result of a secondary immune deficiency?

A 
Malnutrition
Infection (HIV)
Liver diseases
Lymphoproliferative diseases
Splenectomy
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21
Q

What are the functions of the spleen?

A

Sensing blood borne pathogens
Producing antibodies
Removal of opsonized microbes and immune complexes

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22
Q

What blood borne pathogens does the spleen especially sense?

A

Encapsulated bacteria

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23
Q

What antibodies does the spleen produce?

A

IgM

IgG

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24
Q

When does the spleen produce IgM?

A

Acute response

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25
Q

When does the spleen produce IgG?

A

Long term response

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26
Q

How does the spleen remove opsonised microbes and immune complexes?

A

By splenic macrophages

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27
Q

How does someone with a splenectomy present?

A

Increased susceptibility to encapsulated bacteria

Overwhelming post-splenectomy infection

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28
Q

What bacteria are asplenic people susceptible to?

A

Haemophilus influenzae
Streptococcus pneumoiae
Neisseria meningitidis

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29
Q

What is the risk of death from post-splenectomy infection?

A

1-2% risk of death from overwhelming sepsis/meningitis over 15 years

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30
Q

How is a person with a splenectomy managed?

A

Lifelong penicillin prophylaxis
Immunisation against encapsulated bacteria
Medic alert bracelet

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31
Q

What can an increased susceptibility to infections in haematological malignancies be due to?

A

Chemotherapy induced neutropenia
Chemotherapy induced damage to mucosal surfaces
Vascular catheters penetrating innate immune barriers

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32
Q

How should a suspected neutropenia sepsis be treated?

A

As an acute medical emergency, with empiric antibiotics

Assess a patients risk of septic complications

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33
Q

What can an increased loss or catabolism of immune components be due to?

A

Protein losing conditions

Burns

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34
Q

Give two protein losing conditions

A

Nephropathy

Enteropathy

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35
Q

What gives the large spectrum of primary immune deficiencies (PIDs)?

A

Different clinical phenotypes

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36
Q

How many PIDs are there?

A

> 300

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37
Q

What is needed regarding PIDs?

A

Better diagnostic criteria

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38
Q

Why is a better diagnostic criteria needed for PIDs?

A

General failure to recognise and diagnose PIDs

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39
Q

When are most PIDs diagnosed?

A

~8-12 years from onset of symptoms

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40
Q

What % of patients will be 18 years old + when diagnosis is made?

A

> 60%

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41
Q

What % of patients will have permanent tissue/organ damage by the time a diagnosis is made?

A

37%

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42
Q

What can help recognise PIDs?

A

The 10 warning signs

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43
Q

What are the 10 warning signs of PID for children?

A

4 or more new ear infections within 1 year
2 or more serious sinus infections within 1 year
2 or more months on antibiotics with little effect
2 or more pneumonias within 1 year
Failure of an infant to gain weight or grow normally
Recurrent, deep skin or organ abscesses
Persistent thrush in mouth, or fungal infections on skin
Need for intravenous antibiotics to clear infections
Two or more deep-seated infections including septicaemia
A family history of PID

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44
Q

What are the 10 warning signs of PID for adults?

A

2 or more new ear infections in 1 year
2 or more new sinus infections within 1 year in the absence of allergy
One pneumonia per year for more than 1 year
Chronic diarrhoea with weight loss
Recurrent viral infections (colds, herpes, warts, and condyloma)
Recurrent need for intravenous antibiotics to clear infections
Recurrent, deep abscesses of the skin or internal organs
Persistent thrush or fungal infections on skin or elsewhere
Infection with normally harmless tuberculosis-like bacteria
A family history of PID

45
Q

What are the limitations of the 10 warning signs?

A

Lack of population-based evidence that can aid diagnosis
PID patients have different defects/presentations
PID patients with non-infectious manifestations

46
Q

What population based evidence is there regarding the warning signs for PID?

A

Study on children concluded that 96% of PIDs could be diagnosed with the ‘3 warning signs’

47
Q

What are the 3 warning signs that a study says 96% of children with PID can be diagnosed based on?

A

Family history
Failure to thrive
Diagnosis of sepsis treated with IV antibiotics

48
Q

What is the problem with the 3 warning signs when diagnosing PID?

A

It does not take into account PIDs in adults - the largest proportion of people with PID
Found not to be good at diagnosing antibody deficiencies

49
Q

Give 4 examples of deficiencies leading to PIDs?

A

T cells
B cells
Phagocytes
Complement deficiencies

50
Q

How can PID patients have different presentations?

A

Some infections have subtle presentations

51
Q

What PID patients may have a noninfectious manifestation?

A

Autoimmunity
Malignancy
Inflammatory response

52
Q

Does the distribution of malignancies among primary immunodeficiency disorder subgroups vary?

A

Yes

53
Q

How can the types of PID be identified?

A

Age at symptom onset
Types of microbes
Sites of infection

54
Q

What does a PID onset of <6 months highly suggest?

A

A T-cell or phagocyte defect

55
Q

What does a PID onset of >6 months and <5 years often suggest?

A

A B-cell/antibody or phagocyte defect

56
Q

What does a PID onset of > 5 years and later in life usually suggest?

A

A B-cell/antibody/complement or secondary immunodeficiency

57
Q

What bacteria are associated with a complement deficiency PID?

A

Neisseria
Streptococci
Haemophilus influenzae
Other encapsulated bacteria

58
Q

What bacteria are associated with phagocytic defect PIDs?

A

Staph. aureus
Pseudomonas aureginosa
Non-tuberculous mycobacteria

59
Q

What fungi are associated with phagocytic defect PIDs?

A

Candida

Aspergillus

60
Q

What bacteria are associated with antibody deficiency PIDs?

A 
Streptococci
Staphylococci
Haemophilus influenzae
Moraxella catarrhalis 
Pseudomonas aeruginosa
Mycoplasma pneumoniae
61
Q

What viruses are associated with antibody deficiency PIDs?

A

Enteroviruses

62
Q

What protozoa are associated with antibody deficiency PID?

A

Giardia lamblia

63
Q

What bacteria are associated with T cell defect PIDs?

A

Similar to antibody deficiencies, but also include;
Salmonella
Listeria monocytogenes
Non-tuberclous mycobacteria

64
Q

What viruses are associated with T cell defect PIDs?

A

All viruses

65
Q

What fungi are associated with T cell defect PIDs?

A

Candida
Aspergillus
Cryptococcus neoformans
Histoplasma capsulatum

66
Q

What protozoa are associated with T cell defect PIDs?

A

Pneumocystis
Toxoplasma gondii
Cryptosporidium parvum

67
Q

What sites of infection are complement deficiencies associated with?

A

Pyogenic infections

Meningitis/sepsis/arthritis

68
Q

What complement deficiency is associated with pyogenic infections?

A

C3

69
Q

What complement deficiency is associated with meningitis/sepsis/arthritis?

A

C5-9

70
Q

What sites of infection are associated with phagocyte defects?

A

Skin/mucous infections
Deep seated infections
Invasive fungal infections

71
Q

What invasive fungal infection is associated with phagocyte defects?

A

Aspergillosis

72
Q

What sites of infection are associated with antibody deficiencies?

A 
Sino-respiratory infections
Arthopathies
GI infections
Malignancies 
Autoimmunity
73
Q

What will T cell defects lead to if not treated?

A

Failure to thrive and death

74
Q

What sites of infection are associated with T cell defects?

A

Deep skin and tissue abscesses

Opportunistic infections

75
Q

What are the classes of primary immunodeficiencies?

A

Predominantly antibody deficiencies
Combined T and B cell
Phagocytic defects
Other cellular immunodeficincies

76
Q

What % of PIDs are predominantly antibody deficiencies?

A

65%

77
Q

Give 5 predominantly antibody deficiency PIDs

A 
Bruton's disease
Autosomal recessive agammaglobulinaemia
Common variable immunodeficiency (CVID)
Selective IgA deficiency 
IgG subclass deficiency
78
Q

What % of PIDs are combined T and B cells?

A

15%

79
Q

Give 2 combined T and B cell deficiencies

A

SCID

Omenn syndrome

80
Q

What % of PIDs are phagocytic defects?

A

10%

81
Q

Give 3 phagocytic defects

A

Chronic granulomatous disease
Severe congenital neutropenia
Cyclic neutropenia

82
Q

What % of PIDs are other cellular immunodeficiency

A

5-10%

83
Q

Give 4 other cellular immunodeficiencies

A

Wiskott-Aldrich syndrome
DiGeorge syndrome
Hyper IgE syndrome
Ataxia-telangiectasia

84
Q

Why is it important to consider the pattern of family history with PIDs?

A

To help identify any possible X-linked conditions

85
Q

Give an example of an X-linked PID?

A

Bruton’s disease

86
Q

How will patients with CGD often present?

A

With pulmonary aspergilliosis and skin infections

87
Q

How are PIDs managed?

A

Supportive treatment
Specific treatment
Avoid non-essential exposure to radiation
Assess organ damages

88
Q

What are the supportive treatments for PID?

A

Infection prevention
Treat infection promptly and aggressively
Nutritional support
Use UV-irradicated CMVneg blood products only
Avoid live attenuated vaccines in patients with severe PIDs

89
Q

How are infections prevented in PID?

A

Prophylactic antibiotics

90
Q

What nutritional support is given in PID?

A

Vitamins A and D

91
Q

What are the specific treatments for PID?

A

Regular immunoglobulin therapy
Avoid non-essential exposure to radiation
Assess organ damages

92
Q

What is the aim of regular immunoglobulin therapy in PIDs?

A

Get serum IgG > 8g/l

93
Q

How can immunoglobulin be administered?

A

IV or SC

94
Q

What is regular immunoglobulin therapy used for?

A

CVID
XLA
Hyper-IgM syndrome

95
Q

Does aspergillus cause disease?

A

Does not usually cause disease in immunocompetent, but major problem for immunocompromised

96
Q

What is aspergillusosis?

A

An opportunistic fungal infection

97
Q

Where can aspergillus be found?

A

In most buildings

98
Q

What can aspergillus inhalation cause?

A

Can cause aspergillosis in the lungs

99
Q

What happens to the varicella-zoster virus in immunocompetent individuals?

A

It is kept in the latent phase by T cell surveillance after chickenpox infection

100
Q

When may the VSV virus be reactivated?

A

When there is an altered T-cell response or number in immunodeficient patients

101
Q

What does the reactivation of the VSV virus cause?

A

Shingles

102
Q

When does shingles usually appear in healthy patients?

A

Usually in aging patients (thought to be linked to reduced T cell responses)

103
Q

What are the potential sites of infection of S. aureus?

A 
Skin and soft tissue
Respiratory 
Musculoskeletal 
Cardio-vascular
Genitourinary tract
Diseases caused by toxins
104
Q

What skin and soft tissue infections are caused by S. Aureus?

A

Furuncles, carbuncles
Wound infection (traumatic or surgical)
Cellulitis
Impetigo

105
Q

What respiratory infections can be caused by S. Aureus?

A

Pneumonia

106
Q

What musculoskeletal infections can be caused by S. Aureus?

A

Osteomyelitis

Arthritis

107
Q

What cardiovascular infections can be caused by S. Aureus?

A

Endocarditis

Septicaemia

108
Q

What genitourinary infections can be caused by S. Aureus?

A

Renal carbuncle

Lower urinary tract infections

109
Q

What diseases can be caused by S. Aureus toxins?

A

Toxic shock syndrome
Scalded skin syndrome
Food poisioning

[ ESA 3- Infection and Immunity (15 decks)

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