Table 3.

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

TrialTreatment(s)High-risk patientsOutcomes
OverallHigh-risk patients
IMCL-201529
(phase 2, n  =  50) 
Ibrutinib + rituximab Blastoid and Ki-67  ≥  30% excluded
High MIPI  =  38%
TP53 mutations  =  15% 
3-y PFS 93%
3-y OS 92% 
High MIPI and TP53 aberrations associated with inferior PFS and OS.
In TP53 mutated, mPFS of 39 mo vs not reached in wild type. 
NCT0188056730 (phase 2, n  =  50) Ibrutinib + rituximab Blastoid and Ki-67  ≥  50% excluded
High s-MIPI  =  16%
High MIPI-b  =  28%
TP53 aberrations  =  17% 
3-y PFS 87%
3-y OS 94% 
Ki-67 30-50% associated with lower ORR/CR rate.
High s-MIPI associated with inferior PFS.
TP53 aberrations associated with inferior OS but not PFS. 
NCT0521418331 (phase 2, n  =  50) Acalabrutinib + rituximab High s-MIPI  =  22%
Blastoid  =  8%
TP53 aberrations  =  28% (23% with mutations) 
2-y PFS 92%
2-y OS 96% 
Not reported 
OAsIs I35,36 (phase 1/2, n  =  15, treatment-naive cohort) Ibrutinib, obinutuzumab, and venetoclax High MIPI  =  27%
High MIPI-b  =  55%
TP53 mutation  =  13%
del17p deletion  =  40% 
4-y PFS 80%
4-y OS 93% 
Not reported 
ACE-LY-10638 (phase 1b, n  =  21) Acalabrutinib, rituximab, and venetoclax High s-MIPI  =  19%
Blastoid  =  5%
Ki-67  ≥  30%  =  48% 
2-y PFS 54% (95% when censored for COVID-19 deaths)
2-y OS 74% 
Not reported 
BOVen39
(phase 2, n  =  25) 
Zanubrutinib, obinutuzumab, and venetoclax High MIPI  =  68%
Ki-67  ≥  30%  =  67%
Blastoid  =  20%
TP53 mutated  =  100%
Del17p  =  48% 
2-year PFS 72%
2-y PFS 75% 
No significant association between disease-free survival and baseline factors, including Ki-67, morphology, and biallelic TP53 inactivation 
NCT0386318443
(phase 2, n  =  24) 
Acalabrutinib, lenalidomide, and rituximab High MIPI  =  21%
Ki-67  ≥  30%  =  29%
TP53 mutations =  29% 
2-y PFS 87%
2-y OS 100% 
TP53 mutations but not MIPI or Ki-67 were associated with inferior PFS 
NCT0352397544 (phase 1, n  =  29 Venetoclax, lenalidomide, and rituximab High MIPI  =  88%
Blastoid  =  21%
Ki-67  ≥  30%  =  66%
TP53 mutation  =  17% 
2-y PFS 89%
2-y OS 92% 
PFS inferior in patients with TP53 mutation (median: 9 mo vs not reached) 
TrialTreatment(s)High-risk patientsOutcomes
OverallHigh-risk patients
IMCL-201529
(phase 2, n  =  50) 
Ibrutinib + rituximab Blastoid and Ki-67  ≥  30% excluded
High MIPI  =  38%
TP53 mutations  =  15% 
3-y PFS 93%
3-y OS 92% 
High MIPI and TP53 aberrations associated with inferior PFS and OS.
In TP53 mutated, mPFS of 39 mo vs not reached in wild type. 
NCT0188056730 (phase 2, n  =  50) Ibrutinib + rituximab Blastoid and Ki-67  ≥  50% excluded
High s-MIPI  =  16%
High MIPI-b  =  28%
TP53 aberrations  =  17% 
3-y PFS 87%
3-y OS 94% 
Ki-67 30-50% associated with lower ORR/CR rate.
High s-MIPI associated with inferior PFS.
TP53 aberrations associated with inferior OS but not PFS. 
NCT0521418331 (phase 2, n  =  50) Acalabrutinib + rituximab High s-MIPI  =  22%
Blastoid  =  8%
TP53 aberrations  =  28% (23% with mutations) 
2-y PFS 92%
2-y OS 96% 
Not reported 
OAsIs I35,36 (phase 1/2, n  =  15, treatment-naive cohort) Ibrutinib, obinutuzumab, and venetoclax High MIPI  =  27%
High MIPI-b  =  55%
TP53 mutation  =  13%
del17p deletion  =  40% 
4-y PFS 80%
4-y OS 93% 
Not reported 
ACE-LY-10638 (phase 1b, n  =  21) Acalabrutinib, rituximab, and venetoclax High s-MIPI  =  19%
Blastoid  =  5%
Ki-67  ≥  30%  =  48% 
2-y PFS 54% (95% when censored for COVID-19 deaths)
2-y OS 74% 
Not reported 
BOVen39
(phase 2, n  =  25) 
Zanubrutinib, obinutuzumab, and venetoclax High MIPI  =  68%
Ki-67  ≥  30%  =  67%
Blastoid  =  20%
TP53 mutated  =  100%
Del17p  =  48% 
2-year PFS 72%
2-y PFS 75% 
No significant association between disease-free survival and baseline factors, including Ki-67, morphology, and biallelic TP53 inactivation 
NCT0386318443
(phase 2, n  =  24) 
Acalabrutinib, lenalidomide, and rituximab High MIPI  =  21%
Ki-67  ≥  30%  =  29%
TP53 mutations =  29% 
2-y PFS 87%
2-y OS 100% 
TP53 mutations but not MIPI or Ki-67 were associated with inferior PFS 
NCT0352397544 (phase 1, n  =  29 Venetoclax, lenalidomide, and rituximab High MIPI  =  88%
Blastoid  =  21%
Ki-67  ≥  30%  =  66%
TP53 mutation  =  17% 
2-y PFS 89%
2-y OS 92% 
PFS inferior in patients with TP53 mutation (median: 9 mo vs not reached) 

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