VDJ & VJ rearrangement
- VDJ = heavy chain
- VJ = light chain
- randomly inserts nucleotides into DNA sequence at the junctions of V, D, J segments.
- only found in lymphoblasts.
- marker for ALL.
Outer cortex of LN
B cells & follicles
-brutons agammaglobinemia: hypoplastic cortex.
Para-cortex of LN
- paracortex hyperplasia in viral infections.
- hypoplastic in diGeorges
- contains high endothelial venules.
Skin above umbilicus: LN drainage
Lower rectum to anal canal (above pectinate)
Internal iliac LNs
Anal canal below pectinate
Superficial inguinal LNs
Right lymphatic duct
Drains right side of body above diaphragm.
Spleen marginal zone
Where APCs reside and present blood born AGs.
Splenectomy: affect on CBC
cortex = immature T cells. (+) selection here. medulla = mature T cells (MM). (-) selection here.
-bind TCR & CD8
-bind TCR & CD4
required for expression of MHC1. If it was gone, the
active site of the MHC 1 protein would be fucked up
and not work.
Th2 helper Ts
- promote humoral (Ab) immunity (extracellular).
- release IL-4 and IL-5, which promote class switching.
- release IL-10 to shut down Th1 cells. So we dont activate too many T cells, only want Th2 active.
Th1 helper Ts
- intracellular bugs/viruses = cell mediated immunity.
- IL-12 causes formation of Th1 from Th0.
- released IFN-gamma & IL-2
- IFN-gamma shuts down Th2 cells.
FOXP3, CD25, CD3, CD4
-prod anti-infl. cytokines: IL-10 and TGF-β.
- problem w/B cell maturation
- no mature B cells (unlike CVID)
- absent/small LNs & tonsils.
B Cell immunodeficiency mnemonic
SBC = shitty B cells
- Selective IgA deficiency
- Brutons agammaglobinemia
Most common cause of death in DiGeorge?
Congenital heart disease
-tetralogy of fallot, truncus arteriosus, etc.
Autosomal dominant hyper-IgE syndrome
- Deficiency of Th17 cells due to STAT3 mutation.
- impaired recruitment of neutrophils to sites of infection.
-FATED: coarse Facies, cold (noninflamed) staphylococcal Abscesses, retained primary Teeth, inc. IgE, Dermatologic problems (eczema).
- IgA deficiency (also IgG & IgE).
- inc. AFP
Chronic transplant rejection (CTR)
-terminal bronchioles are inflamed/fibrosed.
vanishing bile ducts
vascular fibrosis, glomerulopathy.
Side effects of vincristine
- sensory/motor neuropathies.
- -can have muscle weakness, obtunded reflexes, parasthesias, etc.
- NO BMS.
- enhance neutrophil production.
- aka granulocyte colony-stimulating factor (GCSF)
- xanthine oxidase inhibitor.
- watch out if you’re taking azathioprine/6-MP.
Which hepatitis is DNA?