MDTI - Dale Frank Lectures

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Can you name the MDTI NHL buzzwords?

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

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