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Table 1 Hematologic response and survival outcome of different treatment modalities in AL amyloidosis with different genetic aberrations

From: Genetic pathogenesis of immunoglobulin light chain amyloidosis: basic characteristics and clinical applications

Treatment modalities Arm 1 Arm 2 Patient number (n1/n2) Hematologic response Survival outcome
End point 1 Arm 1/Arm 2 P valuec End point 2 Arm 1/Arm 2 P value
Bortezomib-based [34] t(11;14) Non-(11;14) 44/91 ≥ VGPR 41%/66% < 0.01 5-year OS 46%/72% 0.026
Bortezomib-based [33] t(11;14) Non-(11;14) 64/37 ≥ VGPR 23%/47% 0.02 Median OS 8.7 months/40.7 months 0.05
Bortezomib-based [18] t(11;14) Non-(11;14) 82/89 ≥ VGPR 52%/77% 0.004 Median OS 15.0 months/27.0 months 0.05
Bortezomib-based [18] Trisomies Non trisomies 45/125 ≥ VGPR 72%/65% 0.46 Median OS 14.0 months/38.0 months 0.08
Bortezomib-based [33] High risk aberrationsa Non-high risk aberrations 13/85 ≥ VGPR 67%/26% 0.008 Median OS NR/10.6 months 0.04
MD [35] t(11;14) Non-(11;14) 61/42 ≥ VGPR 18%/22% 0.60 Median OS 38.2 months/17.5 months 0.21
MD [35] Gain of 1q21 Non gain of 1q21 23/77 ≥ VGPR 5%/25% 0.06 Median OS 12.5 months/38.2 months 0.002
MD [35] Del13q14 Non del13q14 36/66 ≥ VGPR 23%/18% 0.78 5-year OS 36%/33% 0.70
MD [35] Hyperdiploidyb Non-hyperdiploidy 15/84 ≥ VGPR 10%/22% 1 5-year OS 40%/36% 0.70
Melphalan-based [18] t(11;14) Non-(11;14) 96/95 ≥ VGPR 41%/54% 0.13 Median OS 23.0 months/26.0 months 0.94
Melphalan-based [18] Trisomies Non trisomies 49/139 ≥ VGPR 39%/52% 0.2 Median OS 15.0 months/32.0 months 0.02
HDM + ASCT [36] t(11;14) Non-(11;14) 72/51 CR 41%/20% 0.02 Median OS NR/93.7 months 0.07
HDM + ASCT [36] Gain of 1q21 Non gain of 1q21 25/91 CR 22%/35% 0.32 Median OS NR/128.8 months 0.93
HDM + ASCT [36] Del13q14 Non del13q14 36/87 CR 21%/37% 0.13 Median OS 128.8 months/NR 0.10
HDM + ASCT [36] Hyperdiploidyb Non-hyperdiploidy 16/95 CR 27%/33% 0.77 Median OS 90.6 months/128.8 months 0.84
HDM + ASCT [36] High risk aberrationsa Non high risk aberrations 9/113 CR 0%/35.2% 0.03 Median OS 47.4 months/NR 0.06
ASCT [18] t(11;14) Non-(11;14) 134/113 ≥ VGPR 70%/78% 0.15 Median OS NR/NR 0.51
ASCT [18] Trisomies Non trisomies 56/186 ≥ VGPR 80%/71% 0.17 Median OS NR/NR 0.98
DD [30] t(11;14) Non-(11;14) 53/32 Median hemEFS 24.3 months/5.5 months < 0.01 Median OS NR/19.3 months 0.07
DD [30] Gain of 1q21 Non gain of 1q21 25/58 Median hemEFS 5.8 months/21.6 months 0.03 Median OS 14.8 months/NR 0.02
DVD [30] t(11;14) Non-(11;14) 23/20 Median hemEFS 19.0 months/10.0 months 0.69 Median OS NR/NR 0.62
DVD [30] Gain of 1q21 Non gain of 1q21 10/30 Median hemEFS 6.8 months/ 22.1 months 0.11 Median OS 9.5 months/NR 0.02
DRD [66] t(11;14) Non-(11;14) 16/15 Median hemEFS 17.3 months/22.6 months > 0.05 –   
DRD [66] Gain of 1q21 Non gain of 1q21 16/15 Median hemEFS 10.9 months/NR < 0.01 –   
IMiD-based [18] t(11;14) Non-(11;14) 8/15 ≥ VGPR 13%/54% 0.04 Median OS 12 months/32 months 0.05
IMiD-based [18] Trisomies Non trisomies 7/17 ≥ VGPR 40%/38% 0.92 Median OS 17 months/23 months 0.93
Venetoclax [44] t(11;14) Non-(11;14) 31/11 ≥ VGPR 78%/30% 0.02 Median OS NR/NR 0.14
  1. MD Melphalan/dexamethasone, HDM high-dose melphalan, ASCT autologous stem cell transplantation, DD daratumumab/dexamethasone, DVD daratumumab/bortezomib/dexamethasone, IMiD immunomodulatory drugs, DRD daratumumab/lenalidomide/dexamethasone, VGPR very good partial response, CR complete response, hemEFS hematologic event-free survival, OS overall survival, PFS progression-free survival, NR not reached
  2. aHigh risk aberrations include t(4;14), t(14;16) and del17p13
  3. bUsing Wuilleme’s criteria.  ≥ VGPR = VGPR + CR
  4. cP values ≦ 0.05 are highlighted in bold
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Experimental Hematology & Oncology

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