Immunodeficiency Part 1 Flashcards

exam 4

1
Q

A 25-year-old woman develops vaginal candidiasis following a course of antibiotics for acne. A small dose of candida extract is injected into the skin of her arm and a red, raised wheal develops at the site in 48 hours. Which one of the following statements is correct:
A) B cell immunodeficiency should be excluded
B) This reaction indicates that the patient has normal cell-mediated immunity
C) IgE antibodies to candida are likely to be increased
D) The patient should have a chest x-ray to exclude tuberculosis
E) Biopsy of this lesion is likely to show immune complexes and complement deposition

A

B) This reaction indicates that the patient has normal cell-mediated immunity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

recurrent infection due to a breakdown in innate immune mechanisms, or humoral or cell-mediated immunity

A

immunodeficiency disorder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

most common defect in immunodeficiency

A

B-cell defect

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

a crucial part of innate immunity is the involvement of

A

neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

functions of neutrophils [3]

A
  1. chemotaxis
  2. phagocytosis
  3. killing
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

neutrophil function;

C3a, C5a and chemokines

A

chemotaxis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

neutrophil function;

IgG and C3b

A

opsonization

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

lysosomal granules contain many bactericidal agents

A

neutrophil killing activity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

low neutrophil count

A

neutropenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

inability to mount a normal inflammatory response

A

defects in neutrophil function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

example of neutropenia

A

patients receiving chemotherapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

IgG deficiency and C3b deficiency

A

defective opsonization

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Chronic granulomatous disease

A

defective neutrophil killing activity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

inability to generate superoxide anion due to a defect in cytochrome b and NADPH oxidase; X-linked

A

Chronic granulomatous disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

chronic granulomatous disease leads to

A

recurrent infection with abscesses of skin, lymph nodes and other organs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Giant lysosomal granules and recurrent infection

A

chediak-higashi syndrome

17
Q

failure neutrophils to emigrate out of vessels toward the antigen and failure of CD8+ cells bind to target cells

A

leukocyte adhesion deficiency (LFA-1)

18
Q

cannot kill straph

A

CGD

19
Q

LFA-1 leads to

A

recurrent bacterial infection and failure to heal wounds–> umbilicus

20
Q

umbilicus

A

LFA-1 deficiency

21
Q

decreased or absent ab resulting in recurrent bacterial infection

A

humoral immuno. disorder

22
Q

absent IgG, IgA, IgM with preB-cells in marrow but no mature B cells; x-linked

A

agammaglobulinemia; Bruton’s

23
Q

Bruton’s Agamma is cuased by a mutation of the ________ leading to

A

Btk gene lading to absent or very small tonsils and lymph nodes

24
Q

Bruton’s tyrosine kinase is important for

A

light chain gene rearrangments

25
Q

someone who has Burton’s what happens to the b-cells in the blood

A

no B-cells (no CD19)

26
Q

Burton’s aggamma. leads to recurrent infections with

A

extracellular pyogenic organims–> staph, strep, hemophilus

27
Q

Tx. for Burton’s

A

intravenous IVIG (IgG) every three weeks

28
Q

common 1/700 and many deficient individuals have no problems

A

IgA deficiency

29
Q

interaction of CD40 and CD40L is crucial for

A

isotype switching

30
Q

IgA deficiency may be associated with _____ or ______leading to developing severe respiratory and GI infections

A

IgG2 or IgG4

31
Q

Mutation of CD40 ligand gene

A

Hyper IgM syndrome

32
Q

what happens with a failure of isotype switching?

A

patients make only IgM and IgD and cannot switch to IgG, IgA or IgE

33
Q

what happens to the lymph nodes from a patient with IgM syndrome

A

no germinal centers

34
Q

B-cells are resent but there is a delay in the production of normal ab

A

transient Hypogammaglobulinemia of childhood

35
Q

Transient hypogamma. has an inability to produce ___ and while the cause is unknown it may be due to a deficiency in

A

IgG and might be due to a deficiency in number and function of helper T-cells

36
Q

usually appears in teenagers or adults and it is due to low serum levels of all immunoglobulins

A

common variable immunodeficiency

37
Q

common variable immunodeficiency defect

A

exact defect is not known but may represent a problem with the maturation of B-cells into plasma cells

38
Q

Tx. for common variable immunodeficiency

A

IVIG