MDTI - Dale Frank Lectures

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Can you name the MDTI NHL buzzwords?

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

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