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Infection > 16. Immunocompromised Host > Flashcards

16. 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
Due to a defect in one or more components of the immune system

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2
Q

Why can a host be immunocompromised?

A

Primary immunodeficiency (congenital) - due to intrinsic gene defect: missing protein, missing cell, non-functional components

Secondary immunodeficiency (acquired) - due to underlying disease/treatment: decreased production/function of immune components, increased loss of catabolism of immune components

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3
Q

What can cause secondary immunodeficiency?

A 
HIV
Chemotherapy
Cancer
Infections
Immunosuppressants (steroids)
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4
Q

When do you suspect an immunodeficiency?

A

Infections defined as SPUR
Severe - IV antibiotics, hospitalisation
Persistent - despite conventional treatment not responding, or takes longer than usual
Unusual - site of infection, type of microorganism
Recurrent

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5
Q

What are the broad categories of immunodeficiencies caused by antibody defects?

A

Defect in B cell development

Defect in antibody production

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6
Q

What are the defects in B cell development?

A

X-linked agammaglobulinaemia (Bruton’s disease)

- cannot produce antibodies

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7
Q

What are the defects in antibody production?

A 
Common variable immunodeficiency
Selective IgA deficiency 
IgG subclass deficiency
Hyper-IgM syndrome - unable to produce IgG
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8
Q

What is the most common defect in antibody production that requires treatment?

A

Common variable immunodeficiency

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9
Q

What is the most common defect in antibody production but is asymptomatic?

A

Selective IgA deficiency

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10
Q

What can happen if immunoglobulin replacement therapy is given to someone with selective IgA deficiency?

A

Patients with this immunodeficiency may develop anti-IgA antibodies so the body rejects the immunoglobulin replacement therapy and has an anaphylactic shock

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11
Q

What are the broad types of immunodeficiency caused by T cell defects?

A

Combined B and T cell defects

T cell defects

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12
Q

What are the types of combined B and T cell defects?

A

Severe combined immunodeficiency (SCID)
Wiskott-Aldrich syndrome
Ataxia telangectasia

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13
Q

What are the T cell defects?

A 
Di George syndrome (thymus) - either no thymus or missing part of thymus so T cells cannot mature
CD3 deficiency
MHC class II deficiencies
MHC class I deficiency
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14
Q

What are the broad types of immunodeficiencies causes by phagocytic defects?

A

Defects in respiratory burst
Defect in fusion of lysosome/phagosome
Defect in neutrophil production and chemotaxis

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15
Q

What is the defect in respiratory burst?

A

Chronic granulomatous disease (CGD)

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16
Q

Wha is the defect in fusion of lysosome/phagosome?

A

Chediak-Higashi syndrome

17
Q

What are the defects in neutrophil production and chemotaxis?

A

Cyclic neutropenia

LAD protein deficiencies

18
Q

What is the most common cause of secondary immunodeficiency?

A

Malnutrition

19
Q

What are the most likely primary immunodeficiency diseases if symptom onset is <6 months?

A

T cell or phagocyte defect

20
Q

What are the most likely primary immunodeficiency diseases if symptom onset is >6 months and <5 years old?

A

B cell antibody or phagocyte defect

21
Q

What are the likely primary immunodeficiency diseases if symptom onset is >5 years old?

A

B cell/antibody/complement or secondary immunodeficiency

22
Q

What are the most common bacteria in complement deficiency?

A

Neisseria, streptococci

23
Q

Which immune defect is giardia lamblia most commonly seen in?

A

Antibody deficiency

24
Q

What is the supportive treatment for PIDs?

A

Infection prevention (prophylactic antimicrobial)
Treat infections promptly and aggressively
Nutritional support
Use UV-irradiated CMVneg blood products only
Avoid live attenuated vaccines

25
Q

What is the specific treatment of PIDs?

A

Regular immunoglobulin therapy

SCID: haematopoietic stem cell therapy

26
Q

What is the goal of immunoglobulin replacement therapy?

A

Serum IgG>8g/l

Life long treatment

27
Q

What conditions is immunoglobulin replacement therapy used in?

A

CVID
XLA (Bruton’s disease)
Hyper-IgM syndrome

28
Q

What are the secondary immune deficiencies caused by?

A

Decreased production of immune components

Increased loss of immune components

29
Q

What can cause decreased production of immune components?

A 
Malnutrition
Infection
Liver diseases
Haematological malignancies
Therapeutic treatment
Splenectomy
30
Q

What can cause increased loss of immune components?

A

Protein-losing conditions (nephropathy, enteropathy)

Burns

Infection (18 decks)

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