Science Quiz / MDTI - Dale Frank Lectures

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QUIZ: Can you name the MDTI NHL buzzwords?

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Prompt
presents at single site, bring patients in early
spread quickly to other mucosal sites but may be treated with antibiotics early
diffuse effacement/psuedofollicles
mott cells
spectrum from monoclonal gammopathy of undetermined significance
proliferation rate is high, Ki67+ 40-90%
10-15% get AIHA caused by benign cells
fish-flesh appearance
morphology (centroblastic/immunoblastic) not important
1/3 of all NHL's
Ki67 100%
most common lymphoid malignancy in childhood
30-50% transform to DLBCL
male to female ratio is 2:1
prolymphocytes/paraimmunoblasts
extranodal involvement uncommon
age>60, LDH levels up, multiple extranodal involvement are all bad prognostic signs
15-30% become prolymphocytic leukemia
hypovolemia due to hypercalcemia
3 cardinal features: small mature lymphos, monocytoid B cells, plasma cells
peripheral blood involvement very common
EBV positive is sometimes seen (not burkitt's!)
BCL2+
express CD56 (NCAM)
uncommon, less than 5 percent
most common leukemia in adults
Prompt
interstitial aggregates
plasma cells account for 10% of cellularity
diagnosis (immunophenotype) of exclusion; BCL2+, CD10-, BCL6-
Does dale frank hate medical students? (T/F)
multifocal, destroys bone
13q/14q32
paratrabecular aggregates
severe immunodeficiency due to paraprotein
MUM1 (IRF4), BCL6, BCL10 positive
Most common NHL
treatment must begin IMMEDIATELY
abdominal (ileocecal) masses more common
huge expansion of marginal zone
t(11:18) common/ also t(1:14) and t(14:18)
CD5+/BCL2+
60% nodal, 40% extranodal in GI (unlike indolent lymphomas)
three separate diseases: nodal, extranodal, splenic
Bence Jones protein
extreme paraprotein production
paraprotein production (sometimes)
50% IgG, 25% IgA
serum hyperviscosity
Ki67 rate is low
starry sky
can evolve or start de novo
aggressive extranodal with CNS involvement
Prompt
pleiomorphic and heterogenous, difficult to predict behavior
not technically a lymphoma, but does involve mature B cells
smudge cells
lipid vacuoles in cells
t(8;14) cMYC translocation
lymphoepithelial lesions
Toughie: tumor inhibiting miR-15a/MiR-16-1 lost with 13q deletion
two main types: germinal center or activated b cell
t(14:18)
flame cell
10% undergo Richter's transformation
three main types: endemic, sporadic, everyone has aids
Observation/Rituximab/CHOP as treatment
deletion 13q/11q/trisomy 12/17p
arises from chronic inflammation (h.pylori, campy, burrelia, HepC?)
BCL6+, CD10+, BCL2-
rouleaux
grading based on centroblast number/nodular vs. diffuse growth
Zap 70+ or Zap 70 -
osteoclast activation leads to hypercalcemia
renal failure (casting) due to paraprotein
really attacks facial bones
85% Bone Marrow involvement
marrow replacement leads to severe anemia
most common lymphoid malignancy in African Americans

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