MDTI - Dale Frank Lectures

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Can you name the MDTI NHL buzzwords?

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multifocal, destroys bone
most common leukemia in adults
EBV positive is sometimes seen (not burkitt's!)
t(8;14) cMYC translocation
presents at single site, bring patients in early
extranodal involvement uncommon
Ki67 rate is low
Ki67 100%
really attacks facial bones
grading based on centroblast number/nodular vs. diffuse growth
age>60, LDH levels up, multiple extranodal involvement are all bad prognostic signs
paratrabecular aggregates
spread quickly to other mucosal sites but may be treated with antibiotics early
30-50% transform to DLBCL
pleiomorphic and heterogenous, difficult to predict behavior
severe immunodeficiency due to paraprotein
Does dale frank hate medical students? (T/F)
proliferation rate is high, Ki67+ 40-90%
diffuse effacement/psuedofollicles
most common lymphoid malignancy in African Americans
interstitial aggregates
most common lymphoid malignancy in childhood
peripheral blood involvement very common
plasma cells account for 10% of cellularity
two main types: germinal center or activated b cell
Observation/Rituximab/CHOP as treatment
express CD56 (NCAM)
50% IgG, 25% IgA
aggressive extranodal with CNS involvement
can evolve or start de novo
3 cardinal features: small mature lymphos, monocytoid B cells, plasma cells
BCL6+, CD10+, BCL2-
85% Bone Marrow involvement
paraprotein production (sometimes)
deletion 13q/11q/trisomy 12/17p
three separate diseases: nodal, extranodal, splenic
Bence Jones protein
Zap 70+ or Zap 70 -
Toughie: tumor inhibiting miR-15a/MiR-16-1 lost with 13q deletion
lymphoepithelial lesions
t(11:18) common/ also t(1:14) and t(14:18)
mott cells
morphology (centroblastic/immunoblastic) not important
three main types: endemic, sporadic, everyone has aids
15-30% become prolymphocytic leukemia
huge expansion of marginal zone
flame cell
osteoclast activation leads to hypercalcemia
Most common NHL
diagnosis (immunophenotype) of exclusion; BCL2+, CD10-, BCL6-
uncommon, less than 5 percent
60% nodal, 40% extranodal in GI (unlike indolent lymphomas)
treatment must begin IMMEDIATELY
not technically a lymphoma, but does involve mature B cells
extreme paraprotein production
serum hyperviscosity
10% undergo Richter's transformation
fish-flesh appearance
marrow replacement leads to severe anemia
lipid vacuoles in cells
10-15% get AIHA caused by benign cells
arises from chronic inflammation (h.pylori, campy, burrelia, HepC?)
starry sky
male to female ratio is 2:1
smudge cells
MUM1 (IRF4), BCL6, BCL10 positive
1/3 of all NHL's
spectrum from monoclonal gammopathy of undetermined significance
abdominal (ileocecal) masses more common
hypovolemia due to hypercalcemia
renal failure (casting) due to paraprotein

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