MCL facts Flashcards
immunophenotype of MCL
CD5, CD19, CD20, sIgM/IgD, FMC7, monoclonal kappa/lambda
CD23 negative
strong CYCLIN D1
SOX-11 positive (negative in non-nodal leukemic type)
Incidence MCL
1:200K
Median age MCL
68-71
Translocation of MCL
T(11:14) Cyclin D1-IgH
In situ MCL
Cyclin D1 positive cells, indolent course
mutations in MCL
CDKN2A- Encodes INK4A and ARF
Aggressive histology
CDK4 amplification- agressive
CCND1 gene mutations- non
nodal MCL
Ibrutinib resistance
Atypical cryptic cyclin D1-positive MCL- t(2:11), t(11:22)
Atypical CKD1 negative - cyclin D2 or cyclin D3- same presntation
Cyclin E- blastoid MCL
Genomic instability—Higher degree of aneuploidy is a feature of aggressive-histology
Epigenetic- complex
genomic and higher degree of DNA methylation are associated with aggressive behavior
GI involvement at baseline in MCL %
40-80%
endoscopy is not mandatory
MIPI
Age
ECOG
LDH
WBC
other prognostic factors besided MIPI in MCL
blastoid or pleomorphic histology
Ki 67 >30%
p53
KMT2D
complex karyotype
good prognostic factors in MCL
SOX11 negative
IGHV mutated
R main benefit in MCL after CIT
LyMA trial
3 year main
PFS benefit without OS
Benefit of R main in older adults with MCL
MCL elderly trial
indefinite main
PFS benefit
other studies had contradicting results
BR+i in MCL
SHINE trial
PFS advantage but not OS
increased death due to cardiac tox
no PFS advantage in high risk pts
trail was withdrawn!!
Chemo free doublet in 1st line MCL
WINDOW-1- phase II Ri
MANGROVE- R zanu
ENRICH- Ri
Chemo free triplets in 1st line MCL
BOVen zanu+ven+ritux (TP53)
AVR, AVO acala+ven+ritux
OAsIs ibrutinib+ven+ritux
