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Flashcards in Immunology Deck (78)
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1

What are SPUR infections? What do they indicate?

Serious, Persistent, Unusual, Recurrent
Indicate immune deficiency

2

What is the main hallmark of immune deficiency?

Recurrent infections

3

Primary immune deficiencies are common. True/False?

False
Rare! Secondary immune deficiencies are common

4

Risk of infection ______ as neutrophil count increases

Decreases

5

How can failure to produce neutrophils arise?

Failure of stem cell differentiation
Failure of neutrophil maturation

6

What is Kostmann syndrome?

Rare autosomal recessive disorder; congenital neutropenia
Clinically presents as (recurrent) infections 2 weeks after birth

7

What is leukocyte adhesion deficiency?

Rare primary immune deficiency where neutrophils fail to bind to endothelial markers - cannot find where infection is!
Genetic defect in CD18

8

What is chronic granulomatous disease?

Failure of oxidative killing due to inability to generate oxygen free radicals

9

Name an important related side effect of anti-TNF therapy

Reactivation of latent TB

10

In congenital neutropenia, neutrophil count is normal. True/False?

False
Low or absent

11

Is there pus formation in congenital neutropenia?

No

12

In leukocyte adhesion deficiency, neutrophil count is low during infection. True/False?

False
It is high

13

Is there pus formation in leukocyte adhesion deficiency?

No

14

In chronic granulomatous disease, neutrophil count is normal. True/False?

True
Would be raised in the acute stage but not once granuloma has formed

15

Is there pus formation in chronic granulomatous disease?

Yes

16

Give examples of definitive management of phagocyte deficiencies

Bone marrow transplant, gene therapy

17

What cells do T cells arise from?

Haemopoetic stem cells in bone marrow

18

Defects in stem cell differentiation in haemopoetic cells causes which fatal condition?

Reticular dysgenesis

19

What is Severe Combined Immunodeficiency (SCID)?

Failure of production of lymphocytes

20

What are some key clinical phenotypes of SCID?

Unwell by 3 months
Diarrhoea
Failure to thrive
Early death

21

Why does SCID present only after 3 months of age?

Maternal IgG protects the infant up to this point

22

In X-linked SCID, which receptor is mutated?

IL2 receptor

23

What is DiGeorge syndrome?

Failed development of the thymus
"Funny looking kid"

24

Which chromosome is deleted in DiG syndrome?

22q11

25

In DiG syndrome, the thymus fails to develop. What is the consequence of this?

Low/no T cells can mature (thus low T cell numbers)

26

What is hypogammaglobulinaemia?

Failure to produce mature B cells

27

What is common variable immunodeficiency?

Low IgG, IgA and IgE
Leads to recurrent infections, often autoimmune

28

Which type of hypersensitivity reaction do allergic diseases come under?

Type 1

29

Define what is meant by allergy

IgE-mediated response to an external antigen

30

Describe the hygiene hypothesis

Decrease in infectious exposure in early life predisposes to increased sensitivity/predisposition to allergic stimuli