**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Jahanzaib Khwaja, University College London Hospital, London

**Main Body of the Abstract**
Introduction:
Traditional staging in systemic AL amyloidosis have focussed on cardiac biomarkers (troponin T, NT-proBNP) and light chain burden (difference in involved and uninvolved free-light chain, dFLC) as the major determinants of patient survival. Recently the functional assessments, global longitudinal strain (GLS%) and 6-min walk distance (6MWD) have also been shown to be prognostic. We assessed the prognostic value of these biomarkers in a uniformly bortezomib-treated patient population.
Methods:
Patients enrolled in a prospective observational study at the United Kingdom National Amyloidosis Centre treated with bortezomib-based regimens from 2010–2019 were analysed. Overall survival (OS) was defined as time from diagnosis to death/last follow-up.
Results:
1298 patients were treated between 2010-2019 with a median age of 67 years (29-89), 2 (1-5) organs involved: 834 (64%) cardiac, 907 (70%) renal involvement. Median dFLC was 189mg/L (0-15989), NTproBNP 1520 ng/L (12-93602), troponin 54 ng/L (0-742), 6MWD 393m (19-700).  219, 447, 433 and 198 were Mayo stage I, II, IIIA, IIIB, respectively. Median OS was 82 months (95% CI 65-110). On univariable analysis the following variables were strongly predictive of survival (p<0.001): dFLC>180mg/L, modified Mayo staging, GLS%, 6MWD>350m. However on multivariable analysis, 6MWD>350m (p=0.001), GLS% (p<0.001) and modified Mayo (p<0.001) remained independently predictive and dFLC>180mg/L (p=0.90) was no longer predictive of overall survival (table 1).
Conclusion:
In the bortezomib-treatment era, GLS%, 6MWD>350m and modified Mayo staging (troponin, NTproBNP) appear independently predictive and dFLC>180mg/L does not appear to be independently predictive of overall survival. Further analysis in the daratumumab era is warranted.



**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Jahanzaib Khwaja, University College London Hospital, London

**Main Body of the Abstract**
**Introduction:**
Traditional staging in systemic AL amyloidosis have focussed on cardiac biomarkers (troponin T, NT-proBNP) and light chain burden (difference in involved and uninvolved free-light chain, dFLC) as the major determinants of patient survival. Recently the functional assessments, global longitudinal strain (GLS%) and 6-min walk distance (6MWD) have also been shown to be prognostic. We assessed the prognostic value of these biomarkers in a uniformly bortezomib-treated patient population.
**Methods:**
Patients enrolled in a prospective observational study at the United Kingdom National Amyloidosis Centre treated with bortezomib-based regimens from 2010–2019 were analysed. Overall survival (OS) was defined as time from diagnosis to death/last follow-up.
**Results:**
1298 patients were treated between 2010-2019 with a median age of 67 years (29-89), 2 (1-5) organs involved: 834 (64%) cardiac, 907 (70%) renal involvement. Median dFLC was 189mg/L (0-15989), NTproBNP 1520 ng/L (12-93602), troponin 54 ng/L (0-742), 6MWD 393m (19-700).  219, 447, 433 and 198 were Mayo stage I, II, IIIA, IIIB, respectively. Median OS was 82 months (95% CI 65-110). On univariable analysis the following variables were strongly predictive of survival (p<0.001): dFLC>180mg/L, modified Mayo staging, GLS%, 6MWD>350m. However on multivariable analysis, 6MWD>350m (p=0.001), GLS% (p<0.001) and modified Mayo (p<0.001) remained independently predictive and dFLC>180mg/L (p=0.90) was no longer predictive of overall survival (table 1).
**Conclusion:**
In the bortezomib-treatment era, GLS%, 6MWD>350m and modified Mayo staging (troponin, NTproBNP) appear independently predictive and dFLC>180mg/L does not appear to be independently predictive of overall survival. Further analysis in the daratumumab era is warranted.



Refining prognostication in systemic AL amyloidosis PA126 (#384)

We invite you to attend the XIX International Symposium on Amyloidosis to be held in Rochester, MN.

This symposium is organized as a hybrid meeting (with the focus on in-person) held from Sunday, May 26 through Thursday, May 30, 2024.

Together with the ISA Board and members of Mayo Clinic, a very attractive scientific hybrid program is being planned. The program will cover a variety of types of systemic amyloidosis: ATTR (hereditary and wild-type), AL (light-chain), AA and less frequent forms. There will be key lectures, specific symposia including those company sponsored, oral presentations and poster sessions of accepted abstracts.

COURSE DESCRIPTION
This program will provide a comprehensive review of state-of-the-art education in systemic amyloidosis: basic science, pathophysiology, diagnosis, current and emerging treatments. Scientific presentations will include competitive oral and poster abstracts. The rapid evolution of scientific discovery and expanding treatment options has created tremendous interest in the field and this meeting will fill and important gap in educating scientists and clinicians.