Dr. Hubbard Exam 1

Front 1

Enumeration of cell types in the whole blood

Back 1

calculation of the number of cells according to shape of cell

Front 2

assessment of innate immunity

Back 2

assessing the function of neutrophils in producing ROS

Front 3

Healthy neutrophils produce ______ while unhealthy cells _____

Back 3

produce ROS while the unhealthy cells don't produce ROS

Front 4

what is the dye used in assessing neutrophils

Back 4

NBT reduction, turn from red to blue
blue cells can produce ROS, positive response
red cells can't produce ROS, negative response

Front 5

in the experiment of neutrophils, what patients were studied

Back 5

neutrophils were taken from a heterozygous carrier of CGD (chronic granulomotous disease)
unaffected polymers showed a color change in NBT
affected polymers showed no color change in NBT, stay red
NADPH once activated in the cell, induce ROS to trigger engulfing microbes

Front 6

ROS helps neutrophil how

Back 6

degrade the engulfed molecule

Front 7

what is CGD

Back 7

autoimmune disease, children born with it missing their NADPH oxidase (or defective) and they can't generate ROS

Front 8

enumerating T cells, how

Back 8

isolation of WBC population by gathering blood, layer on sugar gradient, centrifuge, dark red cells go down --> top layer is lymphocytes. then separate the lymphocytes based on size and density

Front 9

CD4 and CD8 response to mitogens and/or antigens

Back 9

mitogen (from a plant) and recall antigens stimulate proliferation of lymphocytes.
***incubate lymphocytes with mitogen --> bind to sugar moiety on surface of lymphocytes causing it to divide (not Ag dependent)
tetanus, albicans antigen and strepto --> these are recall antigens
one mitogen comes from e coli, gram negative bacteria

Front 10

3H thymidine

Back 10

we incubate culture cells with antigens A and B for days, in the last hours we add 3H thymidine --> incorporated into the DNA, radioactive, the more 3H thymidine that we see, the more cells present

Front 11

what is flow cytometry and how it's used

Back 11

it is measurement of cells florescence and light scattering technique to measure sub populations of cells.

Front 12

intrinsic measures of the flow cytometry

Back 12

based on size and granularity, we measure the type of cell.
incident laser light ---> if side scatter, it is size related (arrow goes up) but proportional to granularity ...lysosomal granules
incident laser light --> forward scatter (passes through the cell, straight) it depends on the size
up (granularity), straight through (size)

Front 13

extrinsic properties with the flow cytometry

Back 13

Anti CD3 Ab are conjugated with fluorochrome green dye. when bind to surface of cell (where CD3 is present) that cell is a T cell.
then the Ab does not bind, no CD3, that's a B cell.
the T cells become green and can be viewed.

Front 14

fluorophore

Back 14

on the Fc tail of Ab, with light, it's activated and projects green light

Front 15

explain the process in the flow cytometer

Back 15

mixed population of cells in the beaker. with it some anti CD4 and anti CD8. through the nozzle, they drop one cell at a time. then we get separate populations in test tubes, some are anti CD4 (CD4 cell bound) and the other anti CD8 (CD8 cell bound)
while the other color is normal cells

Front 16

how is type IV hypersensitivity used in testing anergy

Back 16

using the DTH testing, in which recall antigens are injected SQ , like mumps, candida, tetanus toxoid, trichophyton
the size of induration >5mm is measured 48-72 hours later, erythema (redness) is not measured

Front 17

ELISA (for testing infection and Ab titer)

Back 17

measure patient serum for immunity against infectious agents.
measure protein levels.
Ag is put in well, then Ab is put that will stick to some of the Ag, then a second Ab (goat anti human IgG) will be added with enzyme on the tail. a substrate is added that interacts with the enzyme and turns blue (blue wells are positive)

Front 18

western immuno blot,

Back 18

protein in a gel, the gel is put on a paper, measuring the sized of the proteins (different bands coming down). if the band is attached to patient serum, the pt has the disease

Front 19

ELISA vs Western plot

Back 19

ELISA = hi sensitivity, low specificity
western plot= low sensitivity, hi specificity

Front 20

ELISA in testing HIV

Back 20

ELISA was first used in detecting HIV Ag in patients that are positive, using anti HIV Ab.
it was very sensitive to any Ag present, but not very specific, it binds to any Ag present there and can give false positive

Front 21

what is ANA and how it's used

Back 21

Anti Nuclear Antibody
sensitive but not specific
can detect Ag infected cells by the shape of the florescent cell,
***detecting the type of disease by the shape of the florescence coloring,

Front 22

Reticular Dysgenesis (abnormal organ development)

Back 22

Symptom: complete absence of WBC due to defect in bone marrow and deficiency in stem cells --> rare
Target: bone marrow stem cells
mode of inheritance: autosomal recessive

Front 23

X linked Severe Combined Immunodeficiency Disease (SCID) (bubble boy)

Back 23

Symptoms: mutation on X chromosome. total absence of T cells and NK cells , B cells are present but defective
Target: T and B cells
mode of inheritance: X linked

Front 24

Ataxia-telangiectasia (autosomal recessive)
red eye kid

Back 24

symptoms: mutation in AT gene (involved in DNA repair)
Target: T cells
mode of inheritance: autosomal recessive
possible diseases: lung infection and leukemia and lumphomas

Front 25

Wiskott Aldrich

Back 25

Symptoms: Eczema, thrombocytopenia (low platelets in blood), recurrent respiratory infections, high risk leukemia
Target: T cells
mode of inheritance: X linked

Front 26

2 examples of autosomal recessive immunodeficiency causing enzyme deficiency

Back 26

ADA (Adenosine deaminase) deficiency in gene encoding for the enzyme ADA. death of all T and B cells, Ab are absent.
PNP (purine nucleoside phosphorylase) defect in PNP in purine salvage pathway, decease in T cells
ADA is most common
buildup of adenosine and guanine that cause T and B cells to die

Front 27

what is one case for the treatment of ADA deficiency?

Back 27

gene transfer, for the girl that had ADA deficiency,
gene therapy has been successful in treating this disease

Front 28

Mutations in Rag 1 and Rag 2

Back 28

symptoms: Rag 1 and 2 encode for recombinase enzyme (in VDJ joining in Ig and TCR)
pneumonia and systemic bacterial infections
Target: T and B cells
mode of inheritance: autosomal recessive

Front 29

Di George Syndrome**

Back 29

symptoms: developmental defect in thymus, recurrent infections. congenital heart disease and hypoparathyroid
target: T cells
inheritance: developmental not inherited

Front 30

Nezelof's syndrome

Back 30

symptoms: most die before 2 without treatment. ansent T cells
target: T cells
inheritance: variable inheritance

Front 31

Bare Lymphocyte syndrome

Back 31

symptom; lack class I and II or just I. no positive selection in thymus, no Ag presentation, no CD4+ and CD8+
target: T cells
inheritance: autosomal recessive

Front 32

X linked agammaglobulinemia (Bruton's Disease)

Back 32

symptoms: mutation in gene encoding Bruton's tyrosine kinase, regulator of B cell development. pre B cells do no differentiate. absence of B cells and undetectable Ig
Target:B cells
inheritance: X linked

Front 33

Common Variable Immunodeficiency (CVID)

Back 33

symptoms: normal B cells, fail to differentiate to plasma cells. decrease in IgG, A, M
target: B cells
inheritance: Autosomal recessive

Front 34

IgA deficiency

Back 34

absent IgA.
pulmonary and GI infections

Front 35

Hyper IgM syndrome

Back 35

defect in heavy chain class switch
low IgG, IgA (stays as IgM, does not switch)
X linked

Front 36

Hyper IgE (Jobs Syndrome)

Back 36

hyper IgE,
allergy and asthma

Front 37

Chronic Granulomatous Disease

Back 37

Phagocytic dysfunction
deficinecy in NADPH oxidase, can't make ROS,
X linked and autosomal recessive

Front 38

Chediak-Higashi syndrome (white hair kids)

Back 38

autosomal recessive
phagocytic dysfunction
lysosomes in PMNs don't degranulate, more susceptable to pathogens (can't kill them)
don't have color in hair or skin (melanocytes don't degranulate)

Front 39

Leukocyte Adhesion Molecule deficiency (LAD-1)

Back 39

All WBC can't migrate to site of infection from the blood to tissue
Autosomal recessive

Front 40

Complement deficiencies (C1-C9)

Back 40

Autosomal recessive
deficiencies in the complements
prone to infections, lupus like diseases
recurrent infections

Front 41

Herd Immunity

Back 41

when a critical portion of the community is immunized, most members of the community are protected because there is little chance for an outbreak

Front 42

Active immunization adv and disadv

Back 42

Adv long lasting and high titer
disadv lag time