IgM-related amyloidosis patients treated with ibrutinib
| Pt. . | Lines before AL . | Lymphoma type . | MYD88/ CXCR4 . | Organ involvement (Mayo stage) . | Previous lines . | Best response . | Time from AL diagnosis to ibrutinib treatment, mo . | Ibrutinib response treatment duration, mo . | Adverse events (CTCAE grade), onset since ibrutinib start . | Hematologic toxicity (grade) . | Next line . | Death (cause), survival or follow-up, mo . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | None | WM | MUT/WT | Heart (3), lung | R/B, BRD | PR | 7 | SD/NR, 3 | TIA (3), 1 mo | None | FCR | Yes (stroke), 5 |
| 2 | 1 | WM | MUT/ND | Heart (1) | R/B, CFA | VGPR | 24 | VGPR with OR, 16 | PNP (1), 2 mo | Thrombopenia, anemia (1) | CFA | No, 25 |
| 3 | 1 | WM/MZL | MUT/ND | Kidney, soft tissue | R/CVD, Len/dex | PR | 18 | PD, 2 | Edema (kidney 3), 1 mo | None | ASCT | Yes (sepsis), 11 |
| 4 | 1 | WM | MUT/WT | Heart (ND), soft tissue | R/B | SD/NR | 19 | SD/NR, 6 (continue) | Edema (cardiac 2), 2 mo | Anemia (1) | None | No, 6 |
| 5 | 2 | WM | WT/WT | Heart (3), kidney | CVD | SD/NR | 16 | PR, 9 | PNP (1), diarrhea (1), 1 mo | Thrombopenia (2) | None | Yes (cardiac death), 9 |
| 6 | 1 | WM/MZL | WT/WT | Kidney, liver, PNP | R/B | PR | 22 | SD/NR, 3 | Edema (kidney 1), skin bleeding (1), 2 wk | Anemia (1) | None | Yes (AL progression),9 |
| 7 | None | WM | MUT/ND | Heart (3), soft tissue | R/B | SD/NR | 4 | SD/NR, 2 | Edema (cardiac 3) Atrial fibrillation (3), 2 wk | None | M-dex | Yes (AL progression), 2 |
| 8 | None | WM | ND/ND | Soft tissue | R/B, Len/dex, Vel/dex | PR | 57 | SD/NR, 5 | Atrial fibrillation (2), Pleural effusion (3), 3 mo | None | None | No, 9 |
| Pt. . | Lines before AL . | Lymphoma type . | MYD88/ CXCR4 . | Organ involvement (Mayo stage) . | Previous lines . | Best response . | Time from AL diagnosis to ibrutinib treatment, mo . | Ibrutinib response treatment duration, mo . | Adverse events (CTCAE grade), onset since ibrutinib start . | Hematologic toxicity (grade) . | Next line . | Death (cause), survival or follow-up, mo . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | None | WM | MUT/WT | Heart (3), lung | R/B, BRD | PR | 7 | SD/NR, 3 | TIA (3), 1 mo | None | FCR | Yes (stroke), 5 |
| 2 | 1 | WM | MUT/ND | Heart (1) | R/B, CFA | VGPR | 24 | VGPR with OR, 16 | PNP (1), 2 mo | Thrombopenia, anemia (1) | CFA | No, 25 |
| 3 | 1 | WM/MZL | MUT/ND | Kidney, soft tissue | R/CVD, Len/dex | PR | 18 | PD, 2 | Edema (kidney 3), 1 mo | None | ASCT | Yes (sepsis), 11 |
| 4 | 1 | WM | MUT/WT | Heart (ND), soft tissue | R/B | SD/NR | 19 | SD/NR, 6 (continue) | Edema (cardiac 2), 2 mo | Anemia (1) | None | No, 6 |
| 5 | 2 | WM | WT/WT | Heart (3), kidney | CVD | SD/NR | 16 | PR, 9 | PNP (1), diarrhea (1), 1 mo | Thrombopenia (2) | None | Yes (cardiac death), 9 |
| 6 | 1 | WM/MZL | WT/WT | Kidney, liver, PNP | R/B | PR | 22 | SD/NR, 3 | Edema (kidney 1), skin bleeding (1), 2 wk | Anemia (1) | None | Yes (AL progression),9 |
| 7 | None | WM | MUT/ND | Heart (3), soft tissue | R/B | SD/NR | 4 | SD/NR, 2 | Edema (cardiac 3) Atrial fibrillation (3), 2 wk | None | M-dex | Yes (AL progression), 2 |
| 8 | None | WM | ND/ND | Soft tissue | R/B, Len/dex, Vel/dex | PR | 57 | SD/NR, 5 | Atrial fibrillation (2), Pleural effusion (3), 3 mo | None | None | No, 9 |
The Mayo 2004 staging system was used.
ASCT, autologous stem cell transplantation; BRD, bortezomib, rituximab, dexamethasone; CFA, cyclophosphamide; CTCAE, Common Terminology Criteria for Adverse Events; CVD, cyclophosphamide, bortezomib, dexamethasone; FCR, fludarabine, cyclophosphamide, rituximab; Len/dex, lenalidomide, dexamethasone; M-dex, melphalan, dexamethasone; MUT, mutated; MZL, marginal zone lymphoma; ND, not done; PNP, polyneuropathy; Pt., patient; R/B, rituximab, bendamustine; TIA, transient ischemic attack; Vel/dex, bortezomib, dexamethasone; WT, wild type.
Response criteria: OR, organ response; PD, progressive disease; SD/NR, stable disease/no response.