Immunodeficiency Part 1

Question 1

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

Answer 1

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

Question 2

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

Answer 2

immunodeficiency disorder

Question 3

most common defect in immunodeficiency

Answer 3

B-cell defect

Question 4

a crucial part of innate immunity is the involvement of

Answer 4

neutrophils

Question 5

functions of neutrophils [3]

Answer 5

1. chemotaxis
2. phagocytosis
3. killing

Question 6

neutrophil function;

C3a, C5a and chemokines

Answer 6

chemotaxis

Question 7

neutrophil function;

IgG and C3b

Answer 7

opsonization

Question 8

lysosomal granules contain many bactericidal agents

Answer 8

neutrophil killing activity

Question 9

low neutrophil count

Answer 9

neutropenia

Question 10

inability to mount a normal inflammatory response

Answer 10

defects in neutrophil function

Question 11

example of neutropenia

Answer 11

patients receiving chemotherapy

Question 12

IgG deficiency and C3b deficiency

Answer 12

defective opsonization

Question 13

Chronic granulomatous disease

Answer 13

defective neutrophil killing activity

Question 14

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

Answer 14

Chronic granulomatous disease

Question 15

chronic granulomatous disease leads to

Answer 15

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

Question 16

Giant lysosomal granules and recurrent infection

Answer 16

chediak-higashi syndrome

Question 17

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

Answer 17

leukocyte adhesion deficiency (LFA-1)

Question 18

cannot kill straph

Answer 18

CGD

Question 19

LFA-1 leads to

Answer 19

recurrent bacterial infection and failure to heal wounds–> umbilicus

Question 20

umbilicus

Answer 20

LFA-1 deficiency

Question 21

decreased or absent ab resulting in recurrent bacterial infection

Answer 21

humoral immuno. disorder

Question 22

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

Answer 22

agammaglobulinemia; Bruton’s

Question 23

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

Answer 23

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

Question 24

Bruton’s tyrosine kinase is important for

Answer 24

light chain gene rearrangments

Question 25

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

Answer 25

no B-cells (no CD19)

Question 26

Burton's aggamma. leads to recurrent infections with

Answer 26

extracellular pyogenic organims--> staph, strep, hemophilus

Question 27

Tx. for Burton's

Answer 27

intravenous IVIG (IgG) every three weeks

Question 28

common 1/700 and many deficient individuals have no problems

Answer 28

IgA deficiency

Question 29

interaction of CD40 and CD40L is crucial for

Answer 29

isotype switching

Question 30

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

Answer 30

IgG2 or IgG4

Question 31

Mutation of CD40 ligand gene

Answer 31

Hyper IgM syndrome

Question 32

what happens with a failure of isotype switching?

Answer 32

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

Question 33

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

Answer 33

no germinal centers

Question 34

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

Answer 34

transient Hypogammaglobulinemia of childhood

Question 35

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

Answer 35

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

Question 36

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

Answer 36

common variable immunodeficiency

Question 37

common variable immunodeficiency defect

Answer 37

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

Question 38

Tx. for common variable immunodeficiency

Answer 38

IVIG