CHAPTER: Immuno Flashcards
Mutation, presentation, findings XL agammaglobulinemia
X BTK = X tyrosine kinase = X B cells
**NO Abs (vs CVID with ↓Ig)
> 6 mo, bacterial (IgG), entero + Giardia (IgA) infections
No LNs or tonsils
Presentation, findings IgA def
Mucosal infections
Normal IgG + IgM
If Celiac pt has IgA def - IgG will cause derm hepetiformis
Presentation, findings, complications CVID
Only affects B cells: ↓Ig of all classes (low not no Abs) Same problem as AL-Agamma: 1. X IgG: bacterial 2. X IgA: entero + Giardia ↑AI + lymphoma
Mutation, presentation, findings AD hyper IgE syndrome (Job)
STAT 3 mutation -> X Th17 cells (type Th) ↓Neutrophil to infection sites ↑IgE **Retained primary teeth** Abscess are cold - no neutrophils!
Mutation, presentation, findings ataxia telangiectasia
ATM gene -> bad DNA repair
- Ataxia (cerebellar atrophy) + telang
- ↓ IgA, G, E
- ↑AFP
What T cell disorder might present after BGC admin?
IL 12 R def = AR
If can’t bind IL 12, ↓Th1 –> ↓IFNg
3 causes of SCID
- ADA def (AR)
- IL 2 R defect (XL)
- MHC 2 def = bare lymphocyte syndrome
2 immunodef w/ absent thymic shadow
DiGeorge - X thymus or PT - no T cells
SCID - no thymus or germinal centers in LNs
Mutation, presentation, findings hyper IgM
XL recessive - no CD40L
Can’t class switch B cells after binding Th
Only IgM
Mutation, presentation, findings Wiskott Aldrich
XL recessive WAS gene T cells can't bind cytoskeleton WATER Wisckott Ald TCP Eczema Recurrent infection: ↑IgE, A
Disease w/ delayed umbilical cord sep
LAD - no integrins = can’t bring neutrophils into infection sites
No CD 18 = integrins
↑neutrophils in blood (lose the marginated pool in lungs)
Disease presenting with:
- Albinism
- Peripheral neuropathy
- Infection
Chediak Higashi
MT problem
Giant cells in blood cells @ bone marrow biopsy (pancytopenia b/c nothing can get out)
What T cell mediated acute vs chronic transplant rejection
Acute: CD 8
Chronic: CD 4
Lung, heart, kidney, and liver presentation of chronic rejection
Lung = bronchiolitis obliterans Heart = faster atherosclerosis Kidney = nephropathy Liver = obliterate bile duct
Post blood transfusion pt has low BP, flank pain, hemoglobinuria, and jaundice - what is going on?
You gave them the wrong ABO
Acute hemolytic transfusion rxn
Intra + extravas hemolysis
T2 HST