Table 1.

Selected treatment regimens for patients with WM

StudyAgentN (TN/RR)ORR, %MRR, %VGPR, %PFSAdverse events
Dimopoulos et al44  Cyclophosphamide, D, R 72 (72/0) 83 74 Median: 35 mo Cytopenias, infections, myeloid neoplasms 
Rummel et al45  Bendamustine, R 19 (19/0) NR NR NR Median: 69.5 mo 
R-CHOP 22 (22/0) NR NR NR Median: 28 mo 
Rummel et al27  Bendamustine, R 257 (257/0) 92 88 Median: 65 mo 
Treon et al22  Bortezomib (twice weekly), D, R 23 (23/0) 96 83 22 Median: 66 mo Neuropathy, neutropenia, infections 
Dimopoulos et al46  Bortezomib (weekly), D, R 59 (59/0) 85 58 10 Median: 42 mo 
Treon et al47  Carfilzomib, D, R 31 (31/0) 87 68 35 Median: 44 mo Hyperglycemia, hyperlipasemia 
Castillo et al48  Ixazomib, D, R 26 (26/0) 96 77 15 Median: NR at 22 mo Infections, hyperglycemia 
Treon et al28,29  Ibrutinib 63 (0/63) 91 81 16 5 y: 54% Cytopenias, bleeding, arrhythmias, hypertension 
Treon et al36  Ibrutinib 30 (30/0) 100 83 20 18 mo: 92% 
Dimopoulos et al34  Ibrutinib, R 75 (34/41) 93 73 26 30 mo: 82% 
Owen et al37  Acalabrutinib 106 (14/92) 93 78 8 (IWWM6)
29 (IWWM3) 
24 mo: 90% (TN); 82% (RR) 
StudyAgentN (TN/RR)ORR, %MRR, %VGPR, %PFSAdverse events
Dimopoulos et al44  Cyclophosphamide, D, R 72 (72/0) 83 74 Median: 35 mo Cytopenias, infections, myeloid neoplasms 
Rummel et al45  Bendamustine, R 19 (19/0) NR NR NR Median: 69.5 mo 
R-CHOP 22 (22/0) NR NR NR Median: 28 mo 
Rummel et al27  Bendamustine, R 257 (257/0) 92 88 Median: 65 mo 
Treon et al22  Bortezomib (twice weekly), D, R 23 (23/0) 96 83 22 Median: 66 mo Neuropathy, neutropenia, infections 
Dimopoulos et al46  Bortezomib (weekly), D, R 59 (59/0) 85 58 10 Median: 42 mo 
Treon et al47  Carfilzomib, D, R 31 (31/0) 87 68 35 Median: 44 mo Hyperglycemia, hyperlipasemia 
Castillo et al48  Ixazomib, D, R 26 (26/0) 96 77 15 Median: NR at 22 mo Infections, hyperglycemia 
Treon et al28,29  Ibrutinib 63 (0/63) 91 81 16 5 y: 54% Cytopenias, bleeding, arrhythmias, hypertension 
Treon et al36  Ibrutinib 30 (30/0) 100 83 20 18 mo: 92% 
Dimopoulos et al34  Ibrutinib, R 75 (34/41) 93 73 26 30 mo: 82% 
Owen et al37  Acalabrutinib 106 (14/92) 93 78 8 (IWWM6)
29 (IWWM3) 
24 mo: 90% (TN); 82% (RR) 

D, dexamethasone; MRR, major response rate; NR, not reported; R, rituximab; R-CHOP, rituximab, cyclophosphamide, doxorubucin, vincristine, and prednisone; RR, relapsed/refractory; TN, treatment naive.

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