Immunodeficiency stuff Flashcards
name the four components of the immune function
1) anatomic –> skin, mucosal barriers
2) phagocytes –> neuts, macs
3) cellular immunity
4) humoral immunity –> B cells!
what neutrophil count gives you neutropenia and subsequent infection?
<500 pmns/ul
What are the stages/ counts of HIV/AIDS infection?
early= >500
intermediate= 200-5–
advanced (AIDS)= <200
True false: all the following are messed up in HIV/AIDS?
- CD4 t
- nk
- b
- phagocytes
false. PMNs and macs (phagocytes are okay)
complement is part of _______ immunity.
humoral. stop ignoring that. its not just b cells.
complement deficiency
C2= most common C1-4= classical pathway C5-9= terminal pathway
at which point in the complement pathway do classical, alternative and lectin converge?
C3!
what complements component make up MAC?
C5-9
what infections do you see with:
- C1-C4 defieciency?
- C5-C9 deficiency?
C1-C4= PHYOGENIC infx C5-C9= neisseria
what are some typical presentations of Ab deficiency?
resp infx
encapsulated bacteria –> s. pneumo
enteroviruses
giardia
selective IgA deficiency
MOST COMMON PRIMARY IMMUNODEFICIENCY
- majority asymptomatic because of IgM/IgG
- Airway/ GI infx, Atopy, Autoimmune, Anaphylaxis to IgA products
primary vs secondary immunodeficiency?
Primary: single gene defects
Secondary: malnutrition, HIV/AIDS, age, measles, chronic steroids, chemo, antiTNF, transplant
immunodeficiencies in (2) leukemias?
CLL –> low Ig due to B “arrest”
Multiple myeloma–> high monoclonal IgG, low M/A
CVID (primary immodeficiency)
- recurrent PYOGENIC sinopulm infx –> s pneumo
- chronic diarrhea + GI hyperplasia
- PCP, fungi, mycobacteria, recurrent HSV
- **LOW immunoglobulins due to defective B cell differentiation
Jobs syndrome (hyper IgE)
- AD inheritance –> mutation in STAT3 –> Th17 deficient
- Coarse facies
- Abscesses (cold staph)
- Teeth (retained primary)
- *increased IgE + decrease ifn-y
- Derm probs
CGD mech + susceptible infxs?
- 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
tests for CGD
nitroblue tetrazolium dye NEG
abnl dihydrorhodamine
what are the cat + orgs that take advantage in CGD?
Nocardia psuedo listeria asperg candida e coli staph a serratia
name bugs and things that are susceptible to TMP/SMX proph
listeria nocardia mycobacterium salmonella pneumocystis toxoplasma
TRECs
pieces of DNA cut out during intrathymic T cell receptor gene rearragnement
If TRECs are low, no T cells are being made. What stains should you confirm with on flow?
CD3, gait on:
- CD4/RA+ = naive
- CD4+/RO+= memory cells
Nk stain? Do NK cells express TCR? B cell stain? According to professor lady
No, NK cells= CD3-/CD16+/CD56+
B= CD19+/CD20 (B220)+
there are many defects that can result in SCID. What’s the most common? what are other ways?
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
whats the wonderful underlying reason for which we all chose to go into medicine and why SCID was added to the newborn screen?
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.
clinical presentation of SCID
- failure to thrive
- recurrent pulm infx
- opportunistic infx
- Abesnt thymus (sail sign)
- alopecia, erythroderma, HSM
