Immunodeficiency stuff

Question 1

name the four components of the immune function

Answer 1

1) anatomic –> skin, mucosal barriers
2) phagocytes –> neuts, macs
3) cellular immunity
4) humoral immunity –> B cells!

Question 2

what neutrophil count gives you neutropenia and subsequent infection?

Answer 2

<500 pmns/ul

Question 3

What are the stages/ counts of HIV/AIDS infection?

Answer 3

early= >500
intermediate= 200-5–
advanced (AIDS)= <200

Question 4

True false: all the following are messed up in HIV/AIDS?

• CD4 t
• nk
• b
• phagocytes

Answer 4

false. PMNs and macs (phagocytes are okay)

Question 5

complement is part of _______ immunity.

Answer 5

humoral. stop ignoring that. its not just b cells.

Question 6

complement deficiency

Answer 6

C2= most common
C1-4= classical pathway
C5-9= terminal pathway

Question 7

at which point in the complement pathway do classical, alternative and lectin converge?

Answer 7

C3!

Question 8

what complements component make up MAC?

Answer 8

C5-9

Question 9

what infections do you see with:

• C1-C4 defieciency?
• C5-C9 deficiency?

Answer 9

C1-C4= PHYOGENIC infx
C5-C9= neisseria

Question 10

what are some typical presentations of Ab deficiency?

Answer 10

resp infx
encapsulated bacteria –> s. pneumo
enteroviruses
giardia

Question 11

selective IgA deficiency

Answer 11

MOST COMMON PRIMARY IMMUNODEFICIENCY

• majority asymptomatic because of IgM/IgG
• Airway/ GI infx, Atopy, Autoimmune, Anaphylaxis to IgA products

Question 12

primary vs secondary immunodeficiency?

Answer 12

Primary: single gene defects
Secondary: malnutrition, HIV/AIDS, age, measles, chronic steroids, chemo, antiTNF, transplant

Question 13

immunodeficiencies in (2) leukemias?

Answer 13

CLL –> low Ig due to B “arrest”

Multiple myeloma–> high monoclonal IgG, low M/A

Question 14

CVID (primary immodeficiency)

Answer 14

• recurrent PYOGENIC sinopulm infx –> s pneumo
• chronic diarrhea + GI hyperplasia
• PCP, fungi, mycobacteria, recurrent HSV
• **LOW immunoglobulins due to defective B cell differentiation

Question 15

Jobs syndrome (hyper IgE)

Answer 15

• AD inheritance –> mutation in STAT3 –> Th17 deficient
• Coarse facies
• Abscesses (cold staph)
• Teeth (retained primary)
• *increased IgE + decrease ifn-y
• Derm probs

Question 16

CGD mech + susceptible infxs?

Answer 16

• decrease phago fx: NADPH oxidase deficiency –> decreased o2 radicals + decreased respiratory burst
• increased susceptibility to CATALASE + orgs
• recurrent skin abscesses
• severe/ prolonged PNA
• bone infx

Question 17

tests for CGD

Answer 17

nitroblue tetrazolium dye NEG

abnl dihydrorhodamine

Question 18

what are the cat + orgs that take advantage in CGD?

Answer 18

Nocardia
psuedo
listeria
asperg
candida
e coli
staph a
serratia

Question 19

name bugs and things that are susceptible to TMP/SMX proph

Answer 19

listeria
nocardia
mycobacterium
salmonella
pneumocystis
toxoplasma

Question 20

TRECs

Answer 20

pieces of DNA cut out during intrathymic T cell receptor gene rearragnement

Question 21

If TRECs are low, no T cells are being made. What stains should you confirm with on flow?

Answer 21

CD3, gait on:

• CD4/RA+ = naive
• CD4+/RO+= memory cells

Question 22

Nk stain? Do NK cells express TCR? B cell stain? According to professor lady

Answer 22

No, NK cells= CD3-/CD16+/CD56+

B= CD19+/CD20 (B220)+

Question 23

there are many defects that can result in SCID. What’s the most common? what are other ways?

Answer 23

Most common= X-linked, common gamma chain SCID is part of of multiple cytokine receptors so effects T/B/NK

• defective T/B rearrangement
• defective cytokine signaling
• defective purine metab

Question 24

whats the wonderful underlying reason for which we all chose to go into medicine and why SCID was added to the newborn screen?

Answer 24

Money. It saves money in the future. It also saves lives but that’s only a secondary effect that only occurs when money is saved.

Question 25

clinical presentation of SCID

Answer 25

• failure to thrive
• recurrent pulm infx
• opportunistic infx
• Abesnt thymus (sail sign)
• alopecia, erythroderma, HSM