Immunology Flashcards

1
Q

What is the immune system for?

A

To identify and eliminate micro-organisms and other harmful substances

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2
Q

When should you think about iimmune deficiency with a patient?

A
When a patient presents with SPUR infections: 
Serious
Persistent
Unusual
Recurrent
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3
Q

How do you define a serious infection?

A

Unresponsive to oral antibiotics

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4
Q

How do you define a persistent infection?

A

There is early structural damage

Chronic infections

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5
Q

How do you define an unusual infection?

A

Unusual organisms and sites

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6
Q

How do you define a recurrent infection?

A

Two major or one major and recurrent minor infections in one year

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7
Q

Bar SPUR, what are other features that are suggestive of primary immune deficiency?

A
  • Weight loss/failure to thrive
  • Eczema
  • Chronic diarrhoea
  • Mouth ulceration
  • Family history
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8
Q

How do you classify immunodeficiencies?

A

Primary - rare

Secondary - common, often subtle, often involves more than one component of the immune system

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9
Q

What are the two arms of the immune system?

A

Innate and Adaptive - detect and destroy pathogen in different ways

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10
Q

What cells are involved in the innate immune system?

A

Macrophages, neutrophils, mast cells and NK cells

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11
Q

Which proteins are involved in the innate immune system?

A

Complement, Acute phase proteins and cytokines

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12
Q

What is different about the innate immune system?

A

Recognises structures that are unique to infectious organisms
Essentially identical responses in all individuals

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13
Q

What are the functions off the innate immune system?

A
  • Rapid clearance of micro-organisms
  • Stimulates the acquired (adaptive) immune response
  • Buys time whilst the acquired immune system is mobilised
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14
Q

What are the cells of the acquired immune system?

A

B & T lymphocytes

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15
Q

What are the proteins of the acquired immune system?

A

Antibodies

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16
Q

What is unique about the acquired immune system?

A
  • Repertoire isn’t genetically encoded
  • Acquired as a response to exposure to an antigen
  • Responsive to an unlimited number of molecules
  • Very specific
  • Memory
17
Q

Which cells are the phagocytes?

A

Neutrophils and macrophages

18
Q

What are the functions of phagocytes?

A

Initiation and amplification of inflamm response
Scavenging of cellular and infectious debris
Ingest and kill micro-organisms
Resolution and repair

19
Q

Phagoyctes are particularly important in defence against which pathogens?

A

Bacteria and fungi

20
Q

What are the clinical features of phagocyte deficiencies?

A

Recurrent infections - may affect common and unusual sites

Commonly fungal infections (oral candida)

21
Q

What is Kostmann syndrome?

A

Rare autosomal recessive disorder

22
Q

What is the clinical presentation of Kostmann syndrome?

A

Infections usually within 2 weeks after birth - recurrent bacterial infection, systemic or localised infection

23
Q

What are the non specific features of Kostmann syndrome?

A

fever, irritability, oral ulceration and failure to thrive

24
Q

What are the supportive treatments of Kostmann syndrome?

A

Prophylactic antibiotics and antifungals

25
Q

What is the definitive treatment of Kostmann syndrome?

A

Stem cell transplantation

Granulocyte colony stimulating factor - specific growth factor to assist maturation of neutrophils

26
Q

What is leukocyte adhesion deficiency?

A

Rare primary immunodeficiency that’s caused by a genetic defect in leukocyte integrins (CD18)

27
Q

What is the classical clinical presentation of leukocyte adhesion deficiency?

A

Characterised by marked leukocytosis and localised bacterial infections that are difficult to detect

28
Q

What are the three main types of pathogen recognition receptors?

A

Toll like receptors
Scavenger receptors
Lectin receptors

29
Q

What are opsonins?

A

Molecule that act as binding enhancers for the process of phagocytosis (include complement C3b, IgG antibody and CRP)

30
Q

How do opsonins work?

A

Bind to receptors on phagocyte surface

Phagocytes express Fc receptors - allow binding of antibody that is also bound to antigen

31
Q

What is chronic granulomatous disease?

A

Failure of oxidative killing mechanisms - deficiency of the intracellular killing mechanism of phagocytes - inability to generate oxygen free radicals

32
Q

How are the granulomas formed in chronic granulomatous disease?

A

Inability to clear organisms - excessive inflammation - persistent accumulation of neutrophils, macrophages and lymphocytes

33
Q

What are the features of chronic granulomatous disease?

A
Recurrent deep bacterial infections (especially staphylococcus, aspergillus, pseudomonas cepacia) 
Recurrent fungal infections 
Failure to thrive 
Lymphadenopathy and hepatosplenomegaly 
Granuloma formation