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THE NATURAL HISTORY OF SYSTEMIC AL AMYLOIDOSIS FOLLOWING UPFRONT TREATMENT WITH BORTEZOMIB: AN ANALYSIS OF REAL-WORLD LONGITUDINAL DATA
Author(s): ,
Sriram Ravichandran
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Oliver Cohen
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Steven Law
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Darren Foard
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Marianna Fontana
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Ana Martinez-Nahharo
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Carol Whelan
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Julian Gillmore
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Helen Lachmann
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Sajitha Sachchithanantham
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Shameem Mahmood
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
,
Philip Hawkins
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
Ashutosh Wechalekar
Affiliations:
National Amyloidosis Centre,University College London,London,United Kingdom
EHA Library. Ravichandran S. 06/09/21; 324318; PB1644
Dr. Sriram Ravichandran
Dr. Sriram Ravichandran
Contributions
Abstract

Abstract: PB1644

Type: Publication Only

Session title: Myeloma and other monoclonal gammopathies - Clinical

Background
AL amyloidosis is an incurable disorder. The real natural history of this condition is incompletely understood. 

Aims
In this study, we describe the natural history of the disease based on analysis of real-world longitudinal data.

Methods
All patients seen at the National amyloidosis Centre, the UK between Feb 2010 and Aug 2019 and treated with upfront Bortezomib are included in this analysis. 

Results
1276 patients received 1st line treatment. 26% of patients had died within 12 months of 1st line treatment. 376 (29.5%), 117 (9.2%), 32 (2.5%), 8 (0.6%) and 2 (0.2%) patients had received 2nd, 3rd, 4th, 5th and six lines of treatment, respectively. 77.2% of patients who required more than one line of treatment received the 2nd line within the first two years of 1st line treatment. There was no significant difference in survival from 1st, 2nd, 3rd, and 4th lines of treatment (p=.478). The OS from 1st, 2nd, 3rd, and 4th lines treatment was 56 month, 53 months, 42 months and not reached, respectively (Figure 1). There was no significant difference in TNT from 1st, 2nd, 3rd, and 4th lines of treatment (p=.901). The TNT from 1st, 2nd, 3rd, and 4th lines of treatment was 64 months, 48 months, 36 months, and 44 months, respectively (Figure 1). 

Conclusion
AL Amyloidosis is an incurable disease, and most patients require further treatment. However, even multiply relapsed AL patients have a good outcome and prolonged remission periods.  Prospective trials are needed to refine treatments in the patients with relapsed AL amyloidosis. 

Keyword(s): AL amyloidosis

Abstract: PB1644

Type: Publication Only

Session title: Myeloma and other monoclonal gammopathies - Clinical

Background
AL amyloidosis is an incurable disorder. The real natural history of this condition is incompletely understood. 

Aims
In this study, we describe the natural history of the disease based on analysis of real-world longitudinal data.

Methods
All patients seen at the National amyloidosis Centre, the UK between Feb 2010 and Aug 2019 and treated with upfront Bortezomib are included in this analysis. 

Results
1276 patients received 1st line treatment. 26% of patients had died within 12 months of 1st line treatment. 376 (29.5%), 117 (9.2%), 32 (2.5%), 8 (0.6%) and 2 (0.2%) patients had received 2nd, 3rd, 4th, 5th and six lines of treatment, respectively. 77.2% of patients who required more than one line of treatment received the 2nd line within the first two years of 1st line treatment. There was no significant difference in survival from 1st, 2nd, 3rd, and 4th lines of treatment (p=.478). The OS from 1st, 2nd, 3rd, and 4th lines treatment was 56 month, 53 months, 42 months and not reached, respectively (Figure 1). There was no significant difference in TNT from 1st, 2nd, 3rd, and 4th lines of treatment (p=.901). The TNT from 1st, 2nd, 3rd, and 4th lines of treatment was 64 months, 48 months, 36 months, and 44 months, respectively (Figure 1). 

Conclusion
AL Amyloidosis is an incurable disease, and most patients require further treatment. However, even multiply relapsed AL patients have a good outcome and prolonged remission periods.  Prospective trials are needed to refine treatments in the patients with relapsed AL amyloidosis. 

Keyword(s): AL amyloidosis

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