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RESP > Primary Immunodeficiency 1 > Flashcards

Primary Immunodeficiency 1 Flashcards

1
Q

What aspects of infection can suggest an immunodeficiency? (SPUR)

A
  1. Severe infection
  2. Persistent “
  3. Unusual “
  4. Recurrent “
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2
Q

Primary immunodeficiencies are more common than secondary immunodeficiencies. T/F?

A

False- 2ndary more common

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3
Q

What cells (4) and proteins (3) form the innate immune system?

A
  1. Cells
    - Neutrophils
    - Macrophages
    - NK cells
    - Mast cells
  2. Proteins
    - Complement
    - Cytokine
    - Acute phase protein
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4
Q

Classification of acquired immune system? (2)

A
  1. Cells- B & T lymphocytes

2. Proteins- Antibodies

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5
Q

Two types of phagocytes produced in the body are _ and _.

A

Neutrophils and Macrophages

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6
Q

Phagocyte deficiencies clinically present as _ infections.

A

Recurrent

Common pathogens- Staph, strep
Uncommon- burkholderia, legionella
Mycobacteria
Fungi- candida, aspergillus

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7
Q

What steps are involved in neutrophil action mechanism? Each of these steps can be a focal point of an immunodeficiency. (5)

A
  1. Precursor/phagocyte mobilisation from Bone marrow
  2. Up-regulation of endothelial markers. Neutrophil adhesion + migration into tissues
  3. Pathogen recognition
  4. Pathogen Killing- phagocytosis
  5. Activating other components of immune system
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8
Q

What are the immunodeficiencies of phagocyte production, mobilisation and recruitment? (i.e Stage 1 of the previous question) (2)

A
  1. Failure to produce neutrophils
    - Reticular dysgenesis (SCF lineage problem)
    - After stem cell transplantation
  2. Neutrophil fails to mautre
    - Kostmann’s syndrome- severe congenital neutropenia (GCSF lineage problem)
    - Cyclic neutropenia- episodic neutropenia 4-6 weeks
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9
Q

Kostmann’s syndrome is an _ _ disorder. (genetic)

A

Autosomal recessive

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10
Q

Presentation of Kostmann’s? (5)

A
  1. Infections 2 weeks after birth
  2. Fever
  3. Irritability
  4. Ulceration
  5. Failure to thrive
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11
Q

Treatment for Kostmann? (3)

A
  1. Prophylactic antibiotics/antifungals
  2. Stem cell transplant
  3. GCSF stimulation factor- to help neutrophil maturation
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12
Q

What are 3 pathological features of a neutrophil adhesion problem?

A
  1. Recurrent infections
  2. V high neutrophil blood count
  3. Infection site- Deep tissue.

Neutrophil adhesion deficiencies are rarer, caused by genetic defects

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13
Q

What are the recognition defects see n pathogen recognition deficiencies? (3)

A
  1. Opsonins: may cause defective phagocytosis
  2. Defect in complement/ antibody production
  3. Functional defect of phagocytosis
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14
Q

What disease is linked with failure of pathogen killing?

A

CGD- Chronic granulomatous disease
Inability to generate ROS, hence phagocytosis doesn’t occur.

Failure to clear pathogen- accumulation of neutrophils, macrophages and lymphocytes= granuloma

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15
Q

What are the features of CGD? (4)

A
  1. Recurrent infections
    - Bacterial: staph, pseudomonas, mycoplasmsa
    - Fungal: aspergillus
  2. Failure to thrive
  3. Lymphadenopathy & hepatosplenomegaly
  4. Granuloma formation
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16
Q

What test is used specifically for CGD?

A

NBT- nitroblue tetrazolium- Add neutrophil source of E.Coli to see if the body produces H202- sign of pathogen killing

17
Q

Treatment for CGD? (2)

A
  1. Prophylactics

2. Stem cell transplantation

18
Q

What deficiencies are seen in neutrophils activating other molecules of the immune system?

A

Defect of the IL-12:IFNy pathway.

  • IFNy receptor deficiency
  • IL12 receptor deficiency
  • IL12 deficiency
19
Q

What common infections are found in people with IL-12: IFNye defects? (2)

A
  1. Mycobacterial infection

2. Salmonella

20
Q

What are the useful investigations to assess phagocyte function? (6)

A
  1. Neutrophil count- FBC
  2. Pus presence
  3. Neutrophil adhesion markers
  4. Chemotactic assays
  5. Phagocytosis assays
  6. NBT
21
Q

Give an example of a phrophylactic antibiotic

A

Septrin

RESP (25 decks)

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