Diseases and Poorly Organized Syllabus Materials

Question 1

Influenza

Answer 1

orthomyxoviridae, new viral outbreaks every year, have a dsRNA intermediate in host cells

Question 2

first site of entry: influenza

Answer 2

lung epithelial cells (epithelial attempts prevention via release of fatty acids, anti-microbial peptides, and enzymes)

Question 3

epithelial cell response to infection

Answer 3

IL-1 release (increases local heat, activates cells) and TNF release (increase in vascular permeability, integrin activation fluid release and protein release)

Question 4

WBC

Answer 4

4,000 - 11,000

Question 5

lymphocytes (how many)

Answer 5

1,000 - 4,000

Question 6

APECED

Answer 6

occurs from mutations in AIRE, “so that tissue specific antigens needed to tolerize T cell in the thymus are not expressed”; therefore auto-reactive T cells leave the thymus.

Question 7

mutations in fas/fasl

Answer 7

cause a mutation and a “lymphoproliferative” disorder in which T cells are activated and expand uncontrollably

Question 8

immunodeficiency disease from B cell problems

Answer 8

mutations in Igμ, mutations in the surrogate light chain, mutations in CD79: all three are rare

Question 9

flow cytometry and immunohistochemistry in relation to B cells

Answer 9

can be used to determine the development of B cell tumors

Question 10

B cell depletion therapy

Answer 10

targets CD20, FDA approved in patients with autoimmune diseases

Question 11

Abatacept

Answer 11

CTLA4-Ig: blocks CD80/CD86 - CD28 interactions – for autoimmune disease treatment

Question 12

AID and CD40L deficiencies cause

Answer 12

Hyper IgM syndrome (inability to cause the B cell to class switch) – treated with IVIG

Question 13

CVID

Answer 13

Common Variable Immunodeficiency: from ICOSL/ICOS deficiency (needed for germinal center formation)

Question 14

acute lymphoblastic leukemia

Answer 14

pre-B cell tumor (think VpreB and λ5)

Question 15

chronic lymphocytic leukemia (CD5+)

Answer 15

mature B cell

Question 16

diffuse large b-cell lymphoma

Answer 16

germinal center B-cell tumor

Question 17

multiple myeloma

Answer 17

plasma cell tumor

Question 18

cyclosporin

Answer 18

an immunosuppressive drug that targets Calcineurin

Question 19

FK506

Answer 19

an immunosuppressive drug that targets Calcineurin

Question 20

Bruton’s disease

Answer 20

mutated Btk gene as a result of XLA (X-linked agammaglobulinemia), normal number of pre-B cells, not of (im)mature B-cells or serum Ig (children are found to have Burton’s after 6-12 months when they loose their mothers IgG buffer)

Question 21

people under 2 yrs. or people without a spleen are…

Answer 21

are vulnerable to TI-2 infections

Question 22

selective IgA deficiency

Answer 22

the most common immunodeficiency disease (1/5000), children often present with multiple gastrointestinal/respiratory/ear infections