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CMP > Immunodeficiency > Flashcards

Immunodeficiency Flashcards

1
Q

secondary IDs

A

malnutrition, HIV/AIDS,

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2
Q

primary IDs

A

lymphicyte maturation. combined deficiencies, phagocytic defects, complement defects and immune regulation defects

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3
Q

lymphocyte maturation disorders

A

agammaglobulinemia (Burtons)
SCID

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4
Q

phagocytic disorders

A

chronic granulomatous disorders
leukocyte adhesion defiency

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5
Q

immune regulation disorders

A

IPEX, ALPS and FHL

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6
Q

complement disorders

A

class II: angioedema
Class IV: asymptomatic

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7
Q

Maintenance of peripheral tolerance

A

tTreg; apoptosis; anergy; immune prvileged sites; antigen sequestration

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8
Q

how do immune privilged sites maintain immunosuppresion

A

production of TGFb
lack conventinal lymphatic drainage to avoid infiltration
physical barriers
expression of FAS to induce apoptosis

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9
Q

camptothecin is an inhibitor of

A

topoisomerase

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10
Q

activation of psindle assembly checkpoint

A

using MT inhibitors results in defective spindle-kinetochore attachment

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11
Q

why are cancer stem cells challengin to target

A

replicate slowly, reason for relapse post treatment - residual tumour cells in body

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12
Q

inhibit protein synthesis

A

deaminate key AAs to inhibit protein synthesis (L-asparaginase to inhibit protein synthesis)

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13
Q

inhibit dna rpelication

A

inhibit toposiomerase act (camptothecin) –> induce dsdna breaks and adducts
croslsinking agents (cisplatin) to inducer strand crossing, dsdna breaks in s phase

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14
Q

inhibit dna synthesis

A

induce adduct formation (pyrmidine dimers)
inhibit purine synthesis

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15
Q

inhibit division

A

target tubulin to induce apoptotis
inhibit cyclin/kinase complexes to prevent pro-mitotic signalling

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16
Q

inhibit differentiation

A

retinoic acid to neuroblastoma cells

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17
Q

cros slinking agents

A

cisplatin

18
Q

diagnosis

A

use MIBG = noradrenaline analogue taken up by neuroblastoma cells
labelled w radioactive iodine then injected in body + scan to detect

19
Q

bragg peak

A

protons deposit most energy at end of their range

20
Q

pros of proton ptherpay

A

efficient dna direct damage
if can target precisely –. good bc less damage to nromal cells as release of energy in 1 spot

21
Q

pros of photon beam therapy

A

use in cancer near brain ,spine, heart and other vital organs challenging to target

22
Q

surgery for cancer eradication

A

Remove primary tumours + lymph nodes + limit spread

23
Q

inhibiting kinase pathways

A

ras –> raf –> mek –> erk
erk = tf, uoregulates cyclin D allowing progresison thru cell cycle

24
Q

MEK inhibitor

A

trametinib

25
Q

DDR| characteristics

A

broad and integrated, highly specific & highly sensitive

26
Q

DDR: ssbs signal for

A

BER, PARP protein crucial

27
Q

DDR: dsdna breaks signal for

A

NHEJ or HR, BRCA crcucial

28
Q

alloimmunity

A

immune response made by host against non self anitgens (typically derived from transplanted organs)

29
Q

SCID - ex of geneic defects leaidng to scid

A

mutations affecting:
- thymocyte survival
antigen receptor complex
receptor subunits of cytokine receptors

30
Q

IPEX describe

A

defective FOXP3 gene essential for Treg development; lack of Tregs limits elimination of selfreactive effector T cells

31
Q

treatment of digeroge anomaly

A

thymus transplant to thigh

32
Q

ALPS

A

mutation in Fas or it sligand (death receptor) inhibits apoptosis of selfreactive T cells leading to uncontrolled lymphocyte proliferation

33
Q

FHL

A

defective lytic granule release
lymphocytes release IFNy instead of cytokines leading to uncontrolled macrophage proliferation and phagocytosis –> tissue damage

34
Q

chronic granulomatous disease

A

defective NADPH oxidase, failure of phagocytes to produce ROS (superoxide ions) in ohagosomes required to degrade bacteria

35
Q

leukocyte adhesion deficiency

A

mutation affecting cell surface receptors on phagocytes, limiting their ability to slow down and adhere to epithelium in vessel; no intravasation

36
Q

immunsuppressant drug general mechainsm

A

inhibit mast cell degranulation
reduce dendritic cell activation
reduced secretion of inflam cytokines

37
Q

chronic immunsuppressant use side effects

A

hirsutism, long wound healing, striae

38
Q

AID - main features

A

chronic
self pepertuating
mostly tissue specific

39
Q

classes of AID

A

organ specific - T cell mediated
organ spec - antibody mediated
systemic

40
Q

2 main causes of AID

A

breakdown of self tolerance allowing escape of self reactive lymphocytes
genetic susceptibility (MHC locus)

41
Q

ex of genetic susceptibility gene AID

A

MHC II allele DQ8 in type 1 diabetes

42
Q
A

CMP (1 decks)

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