Patients type | Treatment regimen | Efficacy | Study | Phase | N |
---|---|---|---|---|---|
Patients with WM (Overall) | |||||
TN | Ibrutinib + rituximab | PFS: NR (rate 68%); RR: 76%, PR: 45%; VGPR: 29%; CR: 1%; TTNT: NR | iNNOVATE [113] | 3 | 75 |
R/R | Ibrutinib | RR: 77%; VGPR: 29%; PR: 48% | iNNOVATE [114] | 3 | 31 |
R/R | Ibrutinib | ORR: 90.5%, MRR: 79.4%, 5-year OS: 87% | NCT01614821 [115] | 2 | 63 |
TN | Zanubrutinib | MR: 87.5%; VGPR: 33.3%; PR: 54.2% | BGB-3111-AU003 [175] | 1/2 | 24 |
R/R | Zanubrutinib | MR: 79.6%; VGPR: 49%; PR: 28.6% | BGB-3111-AU003 [175] | 1/2 | 49 |
TN | Zanubrutinib | MR: 64%; VGPR: 27%; PR: 36% | NCT04052854 [176] | 2 | 11 |
R/R | Zanubrutinib | MR 90%; VGPR: 43%; PR: 33% | NCT04052854 [176] | 2 | 30 |
R/R | Zanubrutinib | MR: 69.8%; VGPR: 32.6% | BGB-3111–210 [177] | 2 | 44 |
TN | Zanubrutinib | MR: 21%; PR: 47%; VGPR: 26% | ASPEN [112] | 3 | 19 |
R/R | Zanubrutinib | MR: 16%; PR: 49%; VGPR: 29% | ASPEN [112] | 3 | 83 |
TN | Acalabrutinib | ORR: 93%; MR: 14%; PR: 71%; VGPR: 7% | NCT02180724 [178] | 2 | 14 |
R/R | Acalabrutinib | ORR: 94%; MR: 15%; PR 47%; VGPR: 32% | NCT02180724 [178] | 2 | 92 |
R/R | Orelabrutinib | MR: 80.9%; VGPR: 21.3%; PR: 59.6% | ICP-CL-00105 [179] | 2 | 47 |
WM patients with MYD88 mutation | |||||
MYD88L265PCXCR4WT | Ibrutinib | ORR: 100%; MRR: 91.2% | NCT01614821 [119] | 2 | 34 |
R/R: 34; TN: 2 | Acalabrutinib | ORR: 94%; MRR: 78% | NCT02180724 [178] | 2 | 36 |
MYD88L265PCXCR4WT | Orelabrutinib | MRR: 84.6% | ICP-CL-00105 [179] | 2 | – |
MYD88 mutation | Zanubrutinib vs. ibrutinib | CR + VGPR: 36.3% vs. 25.3% | ASPEN [180] | 3 | 201 |
WM patients with MYD88 wild type | |||||
MYD88WTCXCR4WT | Ibrutinib | ORR: 71.4%; MRR: 28.6% | NCT01614821 [119] | 2 | 7 |
R/R: 13; TN: 1 | Acalabrutinib | ORR: 79%; MRR: 57% | NCT02180724 [178] | 2 | 14 |
MYD88WTCXCR4WT | Orelabrutinib | ORR: 25% | ICP-CL-00105 [179] | 2 | – |
MYD88WT | Zanubrutinib | MRR: 65% (including 34% CR + VGPR) | ASPEN [120] | 3 | 28 |
WM patients with CXCR4 mutation | |||||
MYD88MutCXCR4Mut | Ibrutinib | MRR: 68.2%; VGPR: 9.1%; median PFS: 38% | NCT01614821 [115] | 2 | 2 |
CXCR4 mutation | Zanubrutinib vs. ibrutinib | VGPR: 21% vs. 10% MRR: 79% vs. 65% | ASPEN [120] | 3 | 201 |