immunodeficiencies

Question 1

test: susceptibility of asplenia:

Answer 1

encapsulated extracellular bacteria

Question 2

test: susceptibility of c3 deficiency:

Answer 2

recurrent infection with gram-neg bacteria

Question 3

test: susceptibility of cgd

Answer 3

chronic bacterial and fungal infections, impaired neutrophil response

Question 4

innate deficiencies to know

Answer 4

factor I and factor H

Question 5

BTK affected gene? Immune defect? Susceptibility?

Answer 5

BtK, B cells blocked at pro-B-cell stage, recurrent bacterial infection

Question 6

SCID affected gene? immune defect? Susceptibility?

Answer 6

RAG1 or RAG2, no gene rearrangements in B cells and T cells, all types of infections (no rag1 or 2, no t cells, alot of infections)

Question 7

tap1 or tap2 enable:

Answer 7

you to get peptides in the ER so you can present whats in the cytosol. Mutation causes Bare Lymphocyte syndrome

Question 8

mutation in lambda 5?

Answer 8

no b cells produced

Question 9

Xlinked agammaglobonemia

Answer 9

BTK b cells blocked at pro-B cell stage, results in recurrent bacterial infections

Question 10

aire?

Answer 10

transcription factor that drives the expression of non-thymic proteins in the thymus so you can get a broader negative selection

Question 11

ipex

Answer 11

mut is in foxp3, makes you a regulatory tcell bc you ALWAYS need foxp3

Question 12

whats the danger in repeated t cell stimulation?

Answer 12

start recognizing self too well

Question 13

initial hiv infection includes

Answer 13

making antibodies, spike in cd8 t cell activity, the infections virus lays low in plasma until t cells deplete and begin to drop

Question 14

AFT works to:

Answer 14

rapidly lowers viral load in hiv patients

Question 15

corticosteroids TEST:

Answer 15

inhibit inflammation, inhibit many targets including cytokine production by macrophages, nuclear option

Question 16

azathioprine, (cyclophosphamide) mycophenolate TEST!!!! more than the others
(methotrexate)

Answer 16

inhibit proliferation of lymphocytes by :interfering with DNA synthesis!!! that’s why they’re also used as chemotherapy drugs

Question 17

Cyclosporin A tacrolimu (fk506) TEST

Answer 17

inhibit the calcineurin-dependent activation of NFAT, block il-2 production and proliferation by t cells

Question 18

rapamycin (sirolimus) TEST!!

Answer 18

inhibits proliferation of effector T cells by blocking rictor-dependent mTOR activation, and can’t signal through mTOR, you can’t grow, almost all cells need mTOR for growth, to grow, need more proteins, no mTOR

Question 19

lymphocytes and organ rejection, they’re all:

Answer 19

trying to prevent expansion, preventing activation, preventing killing new organ; block Ca signal, growth pathways rapamycin (mTOR) or DNA synthesis azathioprine, (cyclophosphamide) mycophenolate

Question 20

biologics

Answer 20

have well defined targets, bc we know variable region, constant regions, what pathways they should efect rituximab

Question 21

rituximab

Answer 21

specified against anti-CD20, eliminates B cell

Question 22

natalizumab!! TEST

Answer 22

anti-alpha4 integrin, block lymphocyte buffering, MS

Question 23

omalizumab TEST

Answer 23

anti-IgE removes IgE antibody, chronic asthma

Question 24

Ipillmumab TEST

Answer 24

Anti-CTLA-4, Increases CD4 Tcell responses, metastatic melanoma

Question 25

biologics HIV: TEST

Answer 25

cytokines, type 1 interferon

Question 26

cytokines work by: TEST

Answer 26

TNF-specific mAb and solubel TNFR fusion protein, works on crohn’s RA, arthritis, improves joints, increases risk of TB and inection

Question 27

type 1 interferon TEST

Answer 27

used to treat relapsing MS, causes a decrease in relapse rate, risk of liver toxicity flu-like syndrome

Question 28

malignant diseases treatable with BM transplant:

Answer 28

when you get an allogeneic transplant, you can purify someone else’s BM and infuse it, an autologous transplant you use your own BM after purifying it

Question 29

chimeric antigen receptor (CAR) therapy works by:

Answer 29

take t cells out, insert frankenreceptor, put it back in