Table 2.

Selected frontline trials in MCL incorporating novel agents and CIT with outcomes of high-risk patients highlighted

TrialTreatment(s)High-risk patientsOutcomes
OverallHigh-risk patients
SHINE23
(phase 3, n  =  523) 
BR and RM with ibrutinib or placebo High s-MIPI  =  34%
Blastoid =  9%
TP53 mutated  =  10% 
PFS 81 months (BR + ibrutinib) vs 53 mo (BR) No benefit from ibrutinib in high-risk s-MIPI (HR, 1.02; 95% CI, 0.7-1.5) or mutated TP53 (mPFS, 29 mo vs 11 mo; HR, 0.95; 95% CI, 0.5-1.8).
For blastoid: median PFS 26 mo (95% CI, 6-84) with ibrutinib vs 10 mo (95% CI, 6-44 mo) with placebo. 
ECHO24
(phase 3, n  =  598) 
BR and RM with acalabrutinib or placebo High s-MIPI  =  24%
Ki-67  ≥  30%  =  48%
Blastoid  =  13%
TP53 mutated  =  9%
 
PFS 66 mo (BR + acalabrutinib) vs 50 mo (BR) Not reported 
TRIANGLE25 (phase 3, n  =  870) 3 arms (A, I, A + I)
(A) R-CHOP/R-DHAP(X) → ASCT
(I) Ibrutinib + R-CHOP/ R-DHAP(X) → ibrutinib maintenance
(A+I) Ibrutinib + R-CHOP/ R-DHAP(X) → ASCT → ibrutinib maintenance 
High MIPI  =  15%
Ki-67  ≥  30%  =  32%
Blastoid  =  12%
p53  >  50%  =  14%
High-risk biology = 20% 
3-y FFS:
A + I, 88%
I, 86%
A, 72%
3-y OS:
A + I, 91%
I, 92%
A, 86% 
A ± I vs A:
High MIPI: HR  =  0.58 (98.3% CI, 0-1.32)
Blastoid: HR, 0.59 (0-1.63)
Ki-67  ≥  30%: HR, 0.43 (0-0.83)
p53 expression >50%: HR, 0.14 (0-0.57)
High-risk biology: HR, 0.31 (0-0.78)
A vs I:
High MIPI: HR, 1.86 (98.3% CI, 0-4.25)
Blastoid: HR, 1.01 (0-2.52)
Ki-67  ≥  30%: HR, 2.5 (0-4.78)
p53 expression >50%: HR, 3.24 (0-8.5)
High-risk biology: HR, 2.24 (0-4.85) 
WINDOW-126 (phase 2, n  =  131) Ibrutinib + rituximab → HCVAD/MA x4-8 cycles High MIPI  =  8%
High MIPI-b  =  36%
Blastoid  =  12%
Ki-67  ≥  30%  =  45%
TP53 aberrations 32% 
3-y PFS 79%
3-y OS 95% 
CR rate: TP53-mutated 55% vs 91%
PFS:
Ki-67  ≥  30% (HR, 2.73; P  =  .017)
Low/intermediate MIPI-b (HR. 0.17 and 0.24; P  =  .004)
Classic vs blastoid (HR, 0.24; P  <  .001)
TP53 aberrations (HR, 3.87; P  =  .069) 
WINDOW-227
(phase 2, n  =  50) 
Ibrutinib, rituximab, venetoclax → HCVAD/MA  ×  0-4 cycles High s-MIPI  =  6%
Blastoid  =  12%
TP53 aberrations  =  25%
Ki-67 ≥  30%  =  41% 
3-y PFS 86%
3-y OS 86% 
PFS and OS not significantly different based on s-MIPI and TP53 status
PFS and OS inferior in Ki-67  ≥  50% and blastoid 
WAMM33
(phase 2, n  =  444) 
Rituximab + acalabrutinib → R-DHAX → ASCT Ki-67  >  30%  =  66%
Blastoid  =  9% 
2-y PFS 73%
2-y OS 79% 
Not reported 
VR-BAC28
(phase 2, n  =  54) 
R-BAC + venetoclax Ki-67  ≥  30%  =  63%
Blastoid  =  24%
TP53 aberrations  =  63% 
2-y PFS 58%
2-y OS 66% 
Blastoid morphology and TP53 mutations were associated with inferior outcomes in multivariate analysis. 
TrialTreatment(s)High-risk patientsOutcomes
OverallHigh-risk patients
SHINE23
(phase 3, n  =  523) 
BR and RM with ibrutinib or placebo High s-MIPI  =  34%
Blastoid =  9%
TP53 mutated  =  10% 
PFS 81 months (BR + ibrutinib) vs 53 mo (BR) No benefit from ibrutinib in high-risk s-MIPI (HR, 1.02; 95% CI, 0.7-1.5) or mutated TP53 (mPFS, 29 mo vs 11 mo; HR, 0.95; 95% CI, 0.5-1.8).
For blastoid: median PFS 26 mo (95% CI, 6-84) with ibrutinib vs 10 mo (95% CI, 6-44 mo) with placebo. 
ECHO24
(phase 3, n  =  598) 
BR and RM with acalabrutinib or placebo High s-MIPI  =  24%
Ki-67  ≥  30%  =  48%
Blastoid  =  13%
TP53 mutated  =  9%
 
PFS 66 mo (BR + acalabrutinib) vs 50 mo (BR) Not reported 
TRIANGLE25 (phase 3, n  =  870) 3 arms (A, I, A + I)
(A) R-CHOP/R-DHAP(X) → ASCT
(I) Ibrutinib + R-CHOP/ R-DHAP(X) → ibrutinib maintenance
(A+I) Ibrutinib + R-CHOP/ R-DHAP(X) → ASCT → ibrutinib maintenance 
High MIPI  =  15%
Ki-67  ≥  30%  =  32%
Blastoid  =  12%
p53  >  50%  =  14%
High-risk biology = 20% 
3-y FFS:
A + I, 88%
I, 86%
A, 72%
3-y OS:
A + I, 91%
I, 92%
A, 86% 
A ± I vs A:
High MIPI: HR  =  0.58 (98.3% CI, 0-1.32)
Blastoid: HR, 0.59 (0-1.63)
Ki-67  ≥  30%: HR, 0.43 (0-0.83)
p53 expression >50%: HR, 0.14 (0-0.57)
High-risk biology: HR, 0.31 (0-0.78)
A vs I:
High MIPI: HR, 1.86 (98.3% CI, 0-4.25)
Blastoid: HR, 1.01 (0-2.52)
Ki-67  ≥  30%: HR, 2.5 (0-4.78)
p53 expression >50%: HR, 3.24 (0-8.5)
High-risk biology: HR, 2.24 (0-4.85) 
WINDOW-126 (phase 2, n  =  131) Ibrutinib + rituximab → HCVAD/MA x4-8 cycles High MIPI  =  8%
High MIPI-b  =  36%
Blastoid  =  12%
Ki-67  ≥  30%  =  45%
TP53 aberrations 32% 
3-y PFS 79%
3-y OS 95% 
CR rate: TP53-mutated 55% vs 91%
PFS:
Ki-67  ≥  30% (HR, 2.73; P  =  .017)
Low/intermediate MIPI-b (HR. 0.17 and 0.24; P  =  .004)
Classic vs blastoid (HR, 0.24; P  <  .001)
TP53 aberrations (HR, 3.87; P  =  .069) 
WINDOW-227
(phase 2, n  =  50) 
Ibrutinib, rituximab, venetoclax → HCVAD/MA  ×  0-4 cycles High s-MIPI  =  6%
Blastoid  =  12%
TP53 aberrations  =  25%
Ki-67 ≥  30%  =  41% 
3-y PFS 86%
3-y OS 86% 
PFS and OS not significantly different based on s-MIPI and TP53 status
PFS and OS inferior in Ki-67  ≥  50% and blastoid 
WAMM33
(phase 2, n  =  444) 
Rituximab + acalabrutinib → R-DHAX → ASCT Ki-67  >  30%  =  66%
Blastoid  =  9% 
2-y PFS 73%
2-y OS 79% 
Not reported 
VR-BAC28
(phase 2, n  =  54) 
R-BAC + venetoclax Ki-67  ≥  30%  =  63%
Blastoid  =  24%
TP53 aberrations  =  63% 
2-y PFS 58%
2-y OS 66% 
Blastoid morphology and TP53 mutations were associated with inferior outcomes in multivariate analysis. 

HR, hazard ratio.

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