Impact of early response on outcomes in AL amyloidosis following treatment with frontline Bortezomib

Abstract The outcomes in systemic AL amyloidosis are dependent on the depth of haematologic response. However, there is limited data on the impact of the speed of response on outcomes. Here we report the impact of speed of response in a cohort of AL patients treated with upfront Bortezomib. Patients...

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Main Authors: Sriram Ravichandran, Oliver C. Cohen, Steven Law, Darren Foard, Marianna Fontana, Ana Martinez-Naharro, Carol Whelan, Julian D. Gillmore, Helen J. Lachmann, Sajitha Sachchithanantham, Shameem Mahmood, Philip N. Hawkins, Ashutosh D. Wechalekar
Format: Article
Language:English
Published: Nature Publishing Group 2021-06-01
Series:Blood Cancer Journal
Online Access:https://doi.org/10.1038/s41408-021-00510-7
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author Sriram Ravichandran
Oliver C. Cohen
Steven Law
Darren Foard
Marianna Fontana
Ana Martinez-Naharro
Carol Whelan
Julian D. Gillmore
Helen J. Lachmann
Sajitha Sachchithanantham
Shameem Mahmood
Philip N. Hawkins
Ashutosh D. Wechalekar
author_facet Sriram Ravichandran
Oliver C. Cohen
Steven Law
Darren Foard
Marianna Fontana
Ana Martinez-Naharro
Carol Whelan
Julian D. Gillmore
Helen J. Lachmann
Sajitha Sachchithanantham
Shameem Mahmood
Philip N. Hawkins
Ashutosh D. Wechalekar
author_sort Sriram Ravichandran
collection DOAJ
description Abstract The outcomes in systemic AL amyloidosis are dependent on the depth of haematologic response. However, there is limited data on the impact of the speed of response on outcomes. Here we report the impact of speed of response in a cohort of AL patients treated with upfront Bortezomib. Patients seen from February 2010 until August 2019 are included in the present analysis. 1194 & 1133 patients comprised the ITT and 1-month landmark cohorts. In the landmark cohort, 137 (11.5%), 270 (22.6%), 252 (21.1%) and 352 (31.1%) patients had a CR, VGPR, PR and NR at 1-month. Patients with ≥ VGPR at 1-month had significantly better survival (median not reached; at the end of 1, 2, 5,10 years, 87%/92%, 83%/87%, 68%/72% and 63%/58% of patients in CR/VGPR, respectively, were alive) compared to those with a PR (median OS 60 months) or NR (median OS 32 months) (p < 0.005). At 1-month, patients with CR and iFLC < 20 mg/l had a significantly better survival compared to CR and iFLC > 20 mg/l (p = 0.005). Reaching ≥ VGPR at 1-month significantly improved survival in all Mayo disease stages. In conclusion, patients achieving an early deep haematologic response have a significantly superior survival irrespective of cardiac involvement.
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spelling doaj.art-b337fd1300e6447c92566346cf8407bf2022-12-21T18:51:08ZengNature Publishing GroupBlood Cancer Journal2044-53852021-06-011161910.1038/s41408-021-00510-7Impact of early response on outcomes in AL amyloidosis following treatment with frontline BortezomibSriram Ravichandran0Oliver C. Cohen1Steven Law2Darren Foard3Marianna Fontana4Ana Martinez-Naharro5Carol Whelan6Julian D. Gillmore7Helen J. Lachmann8Sajitha Sachchithanantham9Shameem Mahmood10Philip N. Hawkins11Ashutosh D. Wechalekar12National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)National Amyloidosis Centre, University College London (Royal Free Campus)Abstract The outcomes in systemic AL amyloidosis are dependent on the depth of haematologic response. However, there is limited data on the impact of the speed of response on outcomes. Here we report the impact of speed of response in a cohort of AL patients treated with upfront Bortezomib. Patients seen from February 2010 until August 2019 are included in the present analysis. 1194 & 1133 patients comprised the ITT and 1-month landmark cohorts. In the landmark cohort, 137 (11.5%), 270 (22.6%), 252 (21.1%) and 352 (31.1%) patients had a CR, VGPR, PR and NR at 1-month. Patients with ≥ VGPR at 1-month had significantly better survival (median not reached; at the end of 1, 2, 5,10 years, 87%/92%, 83%/87%, 68%/72% and 63%/58% of patients in CR/VGPR, respectively, were alive) compared to those with a PR (median OS 60 months) or NR (median OS 32 months) (p < 0.005). At 1-month, patients with CR and iFLC < 20 mg/l had a significantly better survival compared to CR and iFLC > 20 mg/l (p = 0.005). Reaching ≥ VGPR at 1-month significantly improved survival in all Mayo disease stages. In conclusion, patients achieving an early deep haematologic response have a significantly superior survival irrespective of cardiac involvement.https://doi.org/10.1038/s41408-021-00510-7
spellingShingle Sriram Ravichandran
Oliver C. Cohen
Steven Law
Darren Foard
Marianna Fontana
Ana Martinez-Naharro
Carol Whelan
Julian D. Gillmore
Helen J. Lachmann
Sajitha Sachchithanantham
Shameem Mahmood
Philip N. Hawkins
Ashutosh D. Wechalekar
Impact of early response on outcomes in AL amyloidosis following treatment with frontline Bortezomib
Blood Cancer Journal
title Impact of early response on outcomes in AL amyloidosis following treatment with frontline Bortezomib
title_full Impact of early response on outcomes in AL amyloidosis following treatment with frontline Bortezomib
title_fullStr Impact of early response on outcomes in AL amyloidosis following treatment with frontline Bortezomib
title_full_unstemmed Impact of early response on outcomes in AL amyloidosis following treatment with frontline Bortezomib
title_short Impact of early response on outcomes in AL amyloidosis following treatment with frontline Bortezomib
title_sort impact of early response on outcomes in al amyloidosis following treatment with frontline bortezomib
url https://doi.org/10.1038/s41408-021-00510-7
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