Infection 9 The Immunocompromised Host Flashcards

1
Q

What is an immunocompromised host?

A

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

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2
Q

How is primary immunodeficiency caused?

A

Congential
Due to intrinsic gene defect - missing cell or protein or non functional components

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3
Q

How is secondary immunodeficiency caused?

A

Acquired
Due to underlying disease/treatment
Decreased production/function of immune cells
Increased loss or catabolism of immune cells

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4
Q

When should you suspect immunodeficiency?

A

SPUR
Severe
Persistent
Unusual
Recurrent

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5
Q

What are the 10 warning signs for primary immunodeficiency in children?

A

4+ new ear infections in 1 year
2+ serious sinus infections in 1 year
2+ months on antibiotics with little effect
2+ pneumonias in 1 year
Failure to gain weight or grow normally
Recurrent, deep skin or organ abscesses
Persistent oral thrush or fungal infection on skin
Need IV antibiotic to clear infection
2+ deep seated infections
Family history of PID

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6
Q

What are the 10 warning signs for primary immunodeficiency in adults?

A

2+ new ear infections in 1 year
2+ serious sinus infections in 1 year
Recurrent pneumonias
Chronic diarrhoea with weight loss
Recurrent, deep skin or organ abscesses
Persistent thrush or fungal infection on skin
Need IV antibiotic to clear infection
Infection with normally harmless bacteria
Family history of PID
Recurrent viral infections

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7
Q

What are the limitations of the 10 warning signs of PID?

A

General use
Different presentations + defects per patient
Patients with non-infections manifestations

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8
Q

What defects can cause immunodeficiency?

A

Antibody defects
T cell defects
Phagocytic defects

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9
Q

Types of defects in antibodies causing immunodeficiency

A

Defect in B cell development
Defect in antibody production

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10
Q

Types of defects in T cells causing immunodeficiency

A

Combined B + T cell defects
T cell defects

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11
Q

Types of phagocytic defects causing immunodeficiency

A

Defects in respiratory burst
Defects in fusion of lysosome/phaagosomes
Defects in neutrophil production + Chemotaxis

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12
Q

What are types of unusual presentation?

A

Site of infection
Type of microorganism

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13
Q

What defect does immunodeficiency onset < 6 months suggest?

A

T cell defect
Phagocyte defect

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14
Q

What defect does immunodeficiency onset 6 months to 5 years suggest?

A

B cell defects
Phagocyte defects

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15
Q

What defect does immunodeficiency onset > 5 years suggest?

A

B cell defect
Secondary immunodeficiency

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16
Q

Types of Management of PID

A

Support treatments
Specific treatment
Comorbidities

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17
Q

Supportive treatment of PID

A
  • Infection prevention - prophylactic antimicrobial
  • Treat infections promptly and aggressively
  • Nutritional support
  • Avoid live vaccines
  • Use UV-irradiated CMV negative blood products only
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18
Q

Specific treatment of PID

A

Regular immunoglobulin therapy
Haematopoietic stem cell therapy

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19
Q

Comorbidities management of PID

A

Autoimmunity + malignancies
Organ damage
Avoid non essential exposure to radiation

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20
Q

What immunodeficiency conditions are caused by defects in antibody production?

A

CVID - Common variable immunodeficiency
Selective IgA deficiency

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21
Q

What form of primary antibody deficiency requires treatment?

A

CVID - common variable immunodeficiency

22
Q

What immunodeficiency conditions are caused by defects in B cell production?

A

X-linked agammaglobinaemia - no B cells

23
Q

What immunodeficiency conditions are caused by defects in B and T cells?

A

SCID - Severe combined immunodeficiency

24
Q

What immunodeficiency conditions are caused by defects inT cells?

A

Di George syndrome

25
Q

What immunodeficiency conditions are caused by defects in respiratory burst?

A

CGD - Chronic granulomatous disease

26
Q

What immunodeficiency conditions are caused by defects in fusion of lysosome/phagosomes?

A

Chediak-Higashi syndrome

27
Q

Presentations of chronic granulomatous disease

A

Pulmonary aspergillosis - long term lung infection
Skin infections
Halo signs in HRCT scan

28
Q

Age of onset which suggests T cell or phagocyte defect

A

< 6 months

29
Q

Age of onset which suggests B cell or phagocyte defect

A

6 months - 5 years

30
Q

Age of onset which suggests B cell defect or secondary immunodeficiency

A

> 5 years

31
Q

What conditions can immunoglobulin replacement therapy be used for?

A

Hyper IgM syndrome
X linked agammaglobinaemia
Common variable immunodeficiency

32
Q

Different formulations of immunoglobulin replacement therapy

A

IvIg - intravenous
ScIg -subcutaneous

33
Q

What causes a decreased production of immune components?

A

Malnutrition
Infections e.g. HIV
Liver diseases
Haematological malignancies
Splenectomy
Therapeutic treatments

34
Q

What causes a increased loss of immune components?

A

Burns
Protein-losing conditions e.g. nephropathy, enteropathy

35
Q

What are tonsils mostly made with?

A

B cells

36
Q

Disadvantage of immunoglobulin replacement therapy

A

Life long treatment

37
Q

Why are patients with haematological malignancies more susceptible to infections?

A
  • Chemotherapy induced neutropenia
  • Chemotherapy induced damage to mucosal barrier
  • Vascular catheter - breach of innate immune response > bacteria can enter
38
Q

Lab investigations when there suspicions of T cell deficiency

A

FBC - lymphocyte count
T cell function tests

39
Q

Lab investigations when there suspicions of antibody/B cell deficiency

A

Immunoglobulins

40
Q

What form of primary antibody efficiency doesn’t require treatment?

A

Selective IgA deficiency

41
Q

What is the most common cause of T cell defect?

A

Di George Syndrome

42
Q

Which long term treatment would you suggest for a patient with primary antibody deficiency?

A

Immunoglobulin replacement therapy

43
Q

What causes X linked agammaglobulinaemia?

A

Defect in B cell development - no B cells

44
Q

What causes common variable immunodeficiency?

A

Defect in antibody production

45
Q

What causes selective IgA deficiency?

A

Defective antibody production

46
Q

What causes hyper IgM syndrome?

A

Defect in antibody production

47
Q

What causes severe combined immunodeficiency?

A

Combined B + T cell defects

48
Q

What causes Di George syndrome?

A

T cell defects

49
Q

What causes chronic granulomatous disease?

A

Defects in respiratory burst
Phagocytic defect

50
Q

What casues Chediak Higashi syndrome

A

Defect i the the fusion of lysosome + phagosomes