Immunology

Question 1

Immunology 1 - Primary Immune deficiencies 1

What are the classifications of immune deficiencies?

Answer 1

Primary: single gene mutations

• >100 primary immune deficiencies now currently are described
• Although they are quite rare, only 1:10,000 live births

Secondary: to some other cause

• Infection, malnutrition, haematological malignancy, drugs
• Often involve more than cone component of the immune system

Physiological: to be expected

• Neonates, pregnancy old age

Question 2

Immunology 1 - Primary Immune deficiencies 1

What are the clinical features suggestive of immunodeficiency?

Answer 2

Everyone gets infections, when you want to worry is when:

• TWO major infections or one major and recurrent minor infections happen in 1 year.
• Chronic infections
• Unusual organisms
• Unusual sites
• Unresponsive to treatment
• Other issues/warning signs:
• Family history
• Young age of presentation
• Failure to thrive

Question 3

Immunology 1 - Primary Immune deficiencies 1

What are the cells and soluble components of the innate immune system?

Answer 3

Question 4

Immunology 1 - Primary Immune deficiencies 1

What are phagocytes?

What kind of receptors do they express?

What are they able to detect?

Answer 4

Have the same response in all individuals, These cells can express cytokine and chemokine receptors, allowing them to detect home signals. They also have genetically encoded receptors to detect pathogens, pattern recognition receptors (Toll Like receptors (TLRs) or mannose receptors). These are called pathogen associated molecular patterns (PAMPS) and include bacterial sugars, DNA and RNA.

They express Fc receptors so can detect immune complexes. They have phagocytic capacity and secrete cytokines and chemokines, regulating immune responses.

Question 5

Immunology 1 - Primary Immune deficiencies 1