MDTI - Dale Frank Lectures

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Can you name the MDTI NHL buzzwords?

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

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