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Immunology > Primary Immunodeficiency (1) > Flashcards

Primary Immunodeficiency (1) Flashcards

1
Q

How is immunodeficiency classified? What causes them?

A
  1. Primary
  2. Secondary
  3. Physiological
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2
Q

What clinical features are suggestive of immunodeficiency?

A

Infection

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3
Q

What are features that suggest primary immune deficiency?

A

-

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4
Q

What are the common lab tests we use to detect primary immunodeficiency?

A

Lab tests

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5
Q

What is the innate immune system made of?

A

-

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6
Q

What are the functions of phagocytes?

A

Functions:

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7
Q

Name the polymorphonuclear cells (granulocytes). Where are they produced? What do they release?

A

-

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8
Q

Name the monocytes of these organs. Where are monocytes produced? Where are they stored? What do they differentiate into?

A

-

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9
Q

Describe the function of macrophages.

A
  1. Macrophages in site of infection.
  2. Endothelial cell activation increases expression of adhesion molecules.
  3. Mobilisation of phagocytes and precursors from bone marrow or within tissues.
  4. Increased neutrophil adhesion and migration into infected tissues.
  5. Phagocytosis of organism.
  6. Oxidative and non-oxidative killing
  7. Macrophages present to T cells
  8. Phagocytes die.
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10
Q

What are the causes of phagocyte deficiency?

A
  1. Failure to produce neutrophils.
  2. Defect of phagocyte migration.
  3. Failure of oxidative killing mechanisms.
  4. Cytokine deficiency
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11
Q

What are the causes of failure of production of neutrophils?

A

-

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12
Q

What are the causes of defects of phagocyte migration?

A

-

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13
Q

What are the causes behind failure of oxidative killing mechanisms?

A

-

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14
Q

How can we investigate chronic granulomatous disease?

A

-

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15
Q

What cytokine deficiency causes phagocyte deficiency? What is the normal process?

A

-

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16
Q

What is the result of having a phagocyte deficiency?

A

-

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17
Q

What tests would we use to detect phagocyte deficiency? What results would we expect?

A

*

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18
Q

How do we treat phagocyte deficiencies?

A

-

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19
Q

What are natural killer cells? Why are they important?

A
  • Lymphocytes
  • Have activating and inhibitory receptors.
  • The inhibitory receptors are very important as they recognise HLA class 1 (or self-HLA) –> prevent inappropriate activation by normal self - overrides activation
  • If a cell is infected, it loses the HLA 1 and the NK cell becomes activated and kills the cell
20
Q

What are the types of natural killer cell deficiencies?

A

-

21
Q

What is the result of having NK cell deficiency?

A

Viral Infection as NK cells kill target cells.

22
Q

What is the treatment of NK cell deficiencies?

A

-

23
Q

What disease does a patient with recurrent infections with high neutrophil count on FBC but no abscess formation have?

A

Leukocyte adhesion deficiency

24
Q

What disease does a patient with recurrent infections with hepatosplenomegaly and abnormal dihydrorhodamine test have?

A

Chronic granulomatous disease.

25
Q

What does a patient with recurrent infections with no neutrophils on FBC have?

A

Kostmann syndrome

26
Q

What does a patient with infection with atypical mycobacterium and normal FBC have?

A

IFN gamma receptor deficiency

27
Q

What does a patient with severe chickenpox and disseminated CMV infection have?

A

Classical NK cell deficiency

28
Q

What is the complement system? What is their function

A

-

29
Q

Name the 3 pathways of complement activation

A

-

30
Q

Describe the classical pathway

A

Adaptive immune response.

31
Q

Describe the MBL pathway.

A

Kicks in early.

32
Q

Describe the alternate pathway

A

Kicks in quite early.

33
Q

What is the significance of C3?

A

-

34
Q

What other roles do complement play in immune response?

A

-

35
Q

What happens if you have complement deficiency?

A

-

36
Q

What happens if you have classical pathway deficiency?

A

Fail to produce complement fragment - no phagocytes - nuclear debris –> autoimmune disorder.

37
Q

What happens if you have MBL deficiency?

A

-

38
Q

What is shown in this image?

A

Meningococcal septicaemia - result of complement deficiency.

39
Q

What can active SLE cause?

A

Low c4 and then low c3

40
Q

What can nephritic factors cause?

A

-

41
Q

How do we investigate the complement pathway

A
  • Function complement test:
  • -CH50 –> classical pathway (1,2,3,4,5-9)
  • -AP50 –> alternative pathway (3, 5-9)
42
Q

What would the Ix show for c1q deficiency, properidin deficiency, C9 deficiency and SLE?

A

Properidin part of alternate pathway

43
Q

How do we manage patients with complement deficiencies?

A

-

44
Q

Question

A

C7 deficiency

45
Q

Question.

A

C1q deficiency

Immunology (10 decks)

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