Table 1.

Selected frontline CIT trials in MCL with outcomes of high-risk patients highlighted

Trial (n)Treatment(s)High-risk patientsOutcomes
Overall populationHigh-risk patients
European MCL Younger (n  =  466)48  R-CHOP/R-DHAP → ASCT
vs
R-CHOP → ASCT 
High MIPI  =  14%
High MIPI-c  =  5%
Ki-67  ≥  30%  =   27%
Blastoid  =  9%
p53  >  50%  =  12% 
R-CHOP/R-DHAP: mTTF  =  8 y; mOS  =  NR
R-CHOP: mTTF  =  4  y; mOS  =  11 y 
Benefit in TTF from HDAC was similar across MIPI and MIPI-c risk groups, Ki-67, morphology, and “tended” to be stronger in p53 overexpression 
LYMA (n  =  240)49,50  R-DHAP/ASCT followed by
rituximab vs
observation 
High MIPI  =  18%
Ki-67  >  30%  =  35%
Blastoid  =  10% 
Rituximab: mEFS  =  NR, 7-y OS  =  83%
Observation: mEFS =  6 y, 7-y OS  =  72% 
High MIPI: mPFS and OS = 3.9 and 4.7 y
 
Retrospective/phase 2 (n  =  88)51  BR/R-HDAC High MIPI  =  19%
Ki-67  >  30%  =  24%
Blastoid/pleomorphic  =  12% 
3-y PFS  =  83%
3-y OS  =  92% 
High MIPI 3-y PFS 76%
Blastoid/pleomorphic 3-y PFS 66%
No association between PFS and Ki-67  >  30% 
Single-center phase 2 (n  =  97)52  R-HCVAD Ki-67  ≥  30%  =  8% Blastoid/pleomorphic  =  15%  MIPI predicted OS in overall and older patient cohorts
Blastoid not associated with EFS or OS 
Nordic MCL2 and MCL3 trials (n  =  321, MCL2  =  159, MCL3  =  162)6,53,54  R-maxi-CHOP + R-HDAC High MIPI  =  22%
Ki-67  ≥  30%  =  42%
Blastoid  =  18%
 
mPFS  =  8 y
mOS  =  12.5 y 
High MIPI: mPFS and mOS  =  2.5 and 4 y
MIPI but not blastoid morphology associated PFS and OS in MVA
TP53 mutation: median PFS and OS  =  0.9 and 1.8 y
Del17 (without TP53 mutation): PFS = 3.1 y and OS = 8 y 
Phase 2 (n =  57)55  R-BAC High MIPI  =  44%
Ki-67 ≥  30%  =  31%
Blastoid/pleomorphic  =  25% 
7-y PFS  =  55%
7-y OS  =  63% 
Morphology strongest factor for PFS in MVA 
Trial (n)Treatment(s)High-risk patientsOutcomes
Overall populationHigh-risk patients
European MCL Younger (n  =  466)48  R-CHOP/R-DHAP → ASCT
vs
R-CHOP → ASCT 
High MIPI  =  14%
High MIPI-c  =  5%
Ki-67  ≥  30%  =   27%
Blastoid  =  9%
p53  >  50%  =  12% 
R-CHOP/R-DHAP: mTTF  =  8 y; mOS  =  NR
R-CHOP: mTTF  =  4  y; mOS  =  11 y 
Benefit in TTF from HDAC was similar across MIPI and MIPI-c risk groups, Ki-67, morphology, and “tended” to be stronger in p53 overexpression 
LYMA (n  =  240)49,50  R-DHAP/ASCT followed by
rituximab vs
observation 
High MIPI  =  18%
Ki-67  >  30%  =  35%
Blastoid  =  10% 
Rituximab: mEFS  =  NR, 7-y OS  =  83%
Observation: mEFS =  6 y, 7-y OS  =  72% 
High MIPI: mPFS and OS = 3.9 and 4.7 y
 
Retrospective/phase 2 (n  =  88)51  BR/R-HDAC High MIPI  =  19%
Ki-67  >  30%  =  24%
Blastoid/pleomorphic  =  12% 
3-y PFS  =  83%
3-y OS  =  92% 
High MIPI 3-y PFS 76%
Blastoid/pleomorphic 3-y PFS 66%
No association between PFS and Ki-67  >  30% 
Single-center phase 2 (n  =  97)52  R-HCVAD Ki-67  ≥  30%  =  8% Blastoid/pleomorphic  =  15%  MIPI predicted OS in overall and older patient cohorts
Blastoid not associated with EFS or OS 
Nordic MCL2 and MCL3 trials (n  =  321, MCL2  =  159, MCL3  =  162)6,53,54  R-maxi-CHOP + R-HDAC High MIPI  =  22%
Ki-67  ≥  30%  =  42%
Blastoid  =  18%
 
mPFS  =  8 y
mOS  =  12.5 y 
High MIPI: mPFS and mOS  =  2.5 and 4 y
MIPI but not blastoid morphology associated PFS and OS in MVA
TP53 mutation: median PFS and OS  =  0.9 and 1.8 y
Del17 (without TP53 mutation): PFS = 3.1 y and OS = 8 y 
Phase 2 (n =  57)55  R-BAC High MIPI  =  44%
Ki-67 ≥  30%  =  31%
Blastoid/pleomorphic  =  25% 
7-y PFS  =  55%
7-y OS  =  63% 
Morphology strongest factor for PFS in MVA 

MIPI-c, Combined MIPI; mEFS, median event-free survival; mOS, median OS; mPFS, median PFS; mTTF, median time to treatment failure; MVA, multivariate analysis; NR, not reached.

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