Immunodeficiency Flashcards

1
Q

Primary vs secondary immunodeficiency:

A

Primary is genetic defect

Secondary is effect of external or breakdown in body systems which then affect the immune system

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2
Q

3 examples of primary immune deficiencies affecting the innate immune system?

A

Chronic Granulomatous disease
Leukocyte adhesion deficiency
Complement deficiency

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3
Q

Describe CGD:

A

NADPH oxidase components defect affecting phagocytic cells

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4
Q

Leukocyte adhesion deficiency:

A

B2 integrin defect affecting neutrophils and monocytes

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5
Q

Describe complement deficiency:

A

Complement gene defects eg C8 cant make MAC

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6
Q

Most profound complement deficiency

A

C3 gene

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7
Q

Which CGD is x-linked?

A

gp91- goes through membrane

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8
Q

3 B cell, 2 T cell and 1 overall primary immunodef:

A

B:
Selective IgA def
Hyper IgM syndrome
X-linked agammaglobulinemia

T cells:
Wiskott-Aldrich syndrome
Digeorge syndrome

SCID

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9
Q

What is selective IgA deficiency? explain 3

A

Decerased IgA or no IgA
But in most people it is asymptomatic
Use poly Ig receptor to take IgM to mucousal surfaces and compensate

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10
Q

What is hyper IgM syndrome? why?

A
Defects such as CD40L  (T cells)defect, or any other genes helping signalling e.g. NEMO
No class switching
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11
Q

What is bruton’s tyrosine kinase defect?

A

X-linked agammaglobulinemia - no Igs

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12
Q

What is the wiskott Aldrich syndrome?

A

WASP defect

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13
Q

What is DiGeorge syndrome?

A

TBX1 gene defect , no thymus or partially developed thymus

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14
Q

Example of defective enzymes in SCID?

A

RAG, Yc, JAK3

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15
Q

Which Immunodeficiencies are X linked? usually

A

CGD gp91
DiGeorge
Agammaglobulinemia
SCID (40%)

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16
Q

SCID most common defect:gene and role

A

mutation on yC on X chrom
essential component of IL2, 4, 7, 9, 15 and 21 recptors
These people usually lack T cells and even if B cells present you need T cells to make Ab

17
Q

5 reasons for secondary Immunodeficiencies:

A
Malnutrition
Loss of cellular and humoural components
Tumours
Cytotoxic drugs and irradiation
Infections
18
Q

2 consequences of malnutrition on the immune system

A

Wont respond to infection or vaccines

19
Q

How do you lose immunological components?

A

Lymphocytes passively lost into intestine in intestinal lymphangiectasia

20
Q

2 infection that cause secondary immunodef:

A

HIV and malaria