immune deficiency/infections

Question 1

what are the 3 types of Abs?

Answer 1

neutralizing, opsonizing, complement activating

Question 2

which Ig can cross the placenta?

Answer 2

IgG

Question 3

where do B cells mature and differentiate?

Answer 3

BM

Question 4

what region is found on the HC but not LC?

Answer 4

D

Question 5

what are processes that contribute to B cell diversity?

Answer 5

recombination, chain pairing, junctional diversity, somatic hypermutation

Question 6

when does negative selection happen for B cells?

Answer 6

before leaving BM

Question 7

where does somatic hypermutation happen?

Answer 7

LN

Question 8

through which receptor do B cells present Ag to Th cell?

Answer 8

MHCII

Question 9

what ligand mediates B/T cell binding?

Answer 9

CD40

Question 10

what are T-independent Ags made of?

Answer 10

polysaccharide, lipid

Question 11

what Ag is produced by T-independent B cells?

Answer 11

IgM

Question 12

what does positive selection in B cells do?

Answer 12

select for V-nt variations that lead to better Ag binding

Question 13

what is the most common PID?

Answer 13

humoral

Question 14

what are the different types of B cell deficiencies?

Answer 14

maturation defect, Ab production defect, crosstalk defect

Question 15

what Ig is impacted by transient hypogammaglobulinemia of infancy?

Answer 15

IgG

Question 16

deficiency in which Ig is the most common?

Answer 16

IgA

Question 17

what infection are pts with selective Ab deficiency at risk for?

Answer 17

Strep pneumoniae (no response to pneumovax)

Question 18

what are examples of encapsulated bacteria?

Answer 18

strep, staph, H influenza, N meningitidis, klebsiella, pseudomonas

Question 19

what are PE signs of agammaglobulinemia?

Answer 19

no LN/tonsils

Question 20

what is the mode of inheritance of agammaglobulinemia?

Answer 20

mostly Xlinked through BTK mutation (some AR)

Question 21

what class of diseases do pts with CVID struggle with?

Answer 21

chronic inflammatory diseases (lung, hep, granulomas, IBD)

Question 22

what is the definition of CVID?

Answer 22

decreased levels of 2 Ig types, poor vaccine response, r/o other causes of deficiency

Question 23

what are hyper IgM pts at risk for?

Answer 23

OI (CMV, pneumocystitis), autoimmunity, malignancy

Question 24

how are pts with humoral deficiencies treated?

Answer 24

Ig replacement, antibiotics (prophylaxis), aggressively tx infections, monitor closely

Question 25

where do T cells mature?

Answer 25

thymus

Question 26

what are the different types of APC?

Answer 26

B cells, dendritic cells, macrophages

Question 27

Which MHC moelcule is used to present to Th cells? Tc?

Answer 27

Th-MHCII | Tc-MHCI

Question 28

what is Th1 responsible for? what molecule does it release?

Answer 28

intracellular microbe killing (stimulate macrophage and Ab) IFN-g

Question 29

what is Th2 responsible for? what molecule does it release?

Answer 29

stimulate eosinophils to fight parasites, also activate macrophage in tissue repair IL4, IgE

Question 30

what is Th17 responsible for? what molecule does it release?

Answer 30

recruit neutrophil and monocytes for autoimmune diseases IL17, 22

Question 31

what is T reg responsible for? what molecule does it release?

Answer 31

immunosuppression IL10, TGFb

Question 32

how do cancer cells evade the immune system?

Answer 32

look like self Ag, no MHCI, secrete immunosuppressive proteins

Question 33

what is MSDS?

Answer 33

defect in IFg and IL12 that cause susceptibility to intracellular organisms

Question 34

what organism causes CMC? What deficiency is this caused by?

Answer 34

candida albacans, Th17 deficiency

Question 35

what is SCID?

Answer 35

combined deficiency due to maternal engraftment or T cell mutations

Question 36

why can't live vaccines be given to pts with SCID?

Answer 36

no T cells, so can contract disease

Question 37

what are examples of live vaccines?

Answer 37

varicella, MMR, rota, flu

Question 38

what is the presentation of Xlinked SCID (common gamma chain defect)?

Answer 38

low T/NK, normal B levels but no Igs (maturation defect)

Question 39

what is the triad of wiskott aldrich disease?

Answer 39

eczema, recurrent infection, thrombocytopenia

Question 40

what is the presentation of DiGeorge syndrome?

Answer 40

absence of thymus, low T cells, cardiac abnormalities, hypocalcemia, cleft palate, learning disability

Question 41

GATA2 deficiency presentation

Answer 41

low monocytes, B cells, CD4 HPV infections (NK deficiency)

Question 42

what is the CD4 count in AIDS-defining illness?

Answer 42

<200

Question 43

what are the tests for HIV disease status?

Answer 43

4th gen test (screening), western blot (confirmation)

Question 44

what is the UN 90-90-90 goal?

Answer 44

90% HIV infected know their stat 90% diagnosed on ARV 90% on ARV have suppressed viral loads

Question 45

how are ARV regimens built?

Answer 45

3 drugs from different classes: 2 NRTIs, one of NNRTI, protease inhibitor, or integrase inhibitor

Question 46

what infections are pts with complement deficiency at risk for? How is this deficiency confirmed?

Answer 46

pyogenic/neisseria infection, or SLE CH50 test

Question 47

how do we test for oxidative burst capacity in CGD?

Answer 47

dihydrorhodamine flow cytometry

Question 48

what are examples of catalase + organisms?

Answer 48

staph, Ecoli, klebsiella, pseudomonas, candida, aspergillus

Question 49

what test determines T cell proliferation rate?

Answer 49

mitogen

Question 50

what drugs are used to tx a pseudomonas infection?

Answer 50

piperacillin, ceftazidime, meropenem, gentamycin, cipro

Question 51

what is the nature of a pseudomonas infection?

Answer 51

green pus in burns or cathethers

Question 52

why can't azoles be used to treat PJP?

Answer 52

no ergosterol

Question 53

what is the presentation of PJP?

Answer 53

fever, dry cough, chest pain (batwing on XR)

Question 54

what is the tx for cryotococcus neoformans infections?

Answer 54

L-amphoterocin, fluconazole

Question 55

what complications are associated with toxoplasma gondii infections?

Answer 55

chorioretinitis, brain abscess

Question 56

what does the halo sign indicate in XR?

Answer 56

pulmonary aspergillosis

Question 57

what are the DDx for mono?

Answer 57

cancer, CMV, toxoplasma gondii

Question 58

seeing the owl eye sign on an IBD biopsy indicates what disease?

Answer 58

CMV infection

Question 59

what do gram - cocci pairs indicate in pt that have undergone a splenectomy?

Answer 59

nesseria menigitis