Presentation during EHA2026:All (e)Poster presentations will be made available as of until Thursday, June 11, 2026, 08:00 CEST and will be accessible for on-demand viewing from June 17 to October 15, 2026 on the Congress platform.
Abstract: EHA-6560 Short: PF816
Title: COMPLETE CARDIAC RESPONSE IMPROVES THE SURVIVAL OF PATIENTS WITH AL AMYLOIDOSIS AND IMPAIRED GLOBAL LONGITUDINAL STRAIN
Type: Poster Presentation
Session title: Myeloma and other monoclonal gammopathies - Clinical
Background:
The main predictor of survival in AL amyloidosis is the severity of cardiac involvement. Global longitudinal strain (GLS) has integrated in the current staging system and can identify an ultra-high-risk population with very poor outcomes (Khwaja et al J Clin Oncol 2025). Patients who achieve complete cardiac response, tend to have excellent outcomes and data regarding GLS and graded cardiac response not available yet.
Aims:
To evaluate the impact of graded cardiac response and baseline GLS on the outcomes of patients with AL amyloidosis and cardiac involvement.
Methods:
The analysis included 167 previously untreated consecutive patients with AL amyloidosis and cardiac involvement with available GLS measured at baseline, before treatment initiation.
Results:
Median age was 67 (43-95), 56% were males, 80% had λ light chain type while 51% had also renal, 26% liver and 26% nerve involvement. Mayo stage distribution was 50%, 25% and 25% for stages 2, 3a and 3b. Initial treatment was bortezomib-based without daratumumab in 42% and daratumumab-based in 57%. Median baseline GLS was -13.4 ([-5.7] - [-24]). GLS was correlated with NTproBNP (r=-0.366, p<0.001) and hsTnT (r=-0.5667, p<0.001), and Mayo stage (r=-0.524, p<0.001), but not with clone characteristics such as bone marrow involvement, or free light chain levels. At 6-month landmark, hematologic response rate was 76% for CR/VGPR, 15% PR and 7% NR/PD. Graded cardiac response rates for carCR, carVGPR, carPR and carNR/PD at 6 months were 5.5%, 18%, 35.5% and 50%, at 12 months was 8%, 28%, 35%, 29% and at 24 months 10%, 41%, 28% and 21%, respectively.
Per baseline GLS levels (≥ -9% vs < -9%) graded cardiac response rates at 6 months were 9.8%, 11.5%, 31% and 47.5%, vs 0%, 21%, 39%, 31% and 40% for CR, VGPR, PR, NR/PD (p=0.034), at 12 months was 19%, 14% , 39.5% and 30% vs 4%, 37%, 28% and 31.5% (p=0.012) and at 24 months was 6%, 23%, 29% and 43% vs 7%, 37% 19% and 37% (p=0.508). Baseline GLS was significantly associated with graded cardiac response. In binary logistic regression analysis lower baseline GLS was significantly associated with increased likelihood of achieving carCR at 6 months (OR 1.53, 95% CI 1.139-2.071, p=0.005) and at 12 months (OR 1.345, 95% CI 1.114-1.624, p=0.002). After adjustment for hematologic response at 6 months, baseline GLS remained independent predictor of achieving carCR at 6 months (OR 1.638, 95% CI 1.166-2.303, p=0.004) and at 12 months (OR 1.330, 95% CI 1.107-.599, p=0.002).
Median OS of the whole cohort was 70 months. In univariate analysis GLS < -9% was associated with improved survival (median OS not reached vs 67.7 months; HR 0.561, 95%CI 0.326-0.968, p=0.038). Among patients with GLS >-9, achievement of complete cardiac response at 12 and 24 months was associated with longer OS (Figure 1), while there was no significant difference at 6 months for patients with GLS < -9%. Estimated 2-year survival rate per graded cardiac response at 6 months was 74%, 92% and 100% (p=0.042) for carNR, carPR and carVGPR for patients with impaired GLS (≥-9%) and 73%, 95%, 100% and 100%(p=0.135) for carNR, carPR, carVGPR and carCR.
Summary/Conclusion:
In patients with cardiac AL amyloidosis GLS≥-9% at baseline is associated with lower probability of achieving complete cardiac response independently of hematologic response. However, achievement of carCR is associated with improved outcomes of patients with impaired GLS and this highlights the importance of very effective anti-clonal treatments.
Keyword(s): Free light chain | AL amyloidosis | Plasma cells
Presentation during EHA2026:All (e)Poster presentations will be made available as of until Thursday, June 11, 2026, 08:00 CEST and will be accessible for on-demand viewing from June 17 to October 15, 2026 on the Congress platform.
Abstract: EHA-6560 Short: PF816
Title: COMPLETE CARDIAC RESPONSE IMPROVES THE SURVIVAL OF PATIENTS WITH AL AMYLOIDOSIS AND IMPAIRED GLOBAL LONGITUDINAL STRAIN
Type: Poster Presentation
Session title: Myeloma and other monoclonal gammopathies - Clinical
Background:
The main predictor of survival in AL amyloidosis is the severity of cardiac involvement. Global longitudinal strain (GLS) has integrated in the current staging system and can identify an ultra-high-risk population with very poor outcomes (Khwaja et al J Clin Oncol 2025). Patients who achieve complete cardiac response, tend to have excellent outcomes and data regarding GLS and graded cardiac response not available yet.
Aims:
To evaluate the impact of graded cardiac response and baseline GLS on the outcomes of patients with AL amyloidosis and cardiac involvement.
Methods:
The analysis included 167 previously untreated consecutive patients with AL amyloidosis and cardiac involvement with available GLS measured at baseline, before treatment initiation.
Results:
Median age was 67 (43-95), 56% were males, 80% had λ light chain type while 51% had also renal, 26% liver and 26% nerve involvement. Mayo stage distribution was 50%, 25% and 25% for stages 2, 3a and 3b. Initial treatment was bortezomib-based without daratumumab in 42% and daratumumab-based in 57%. Median baseline GLS was -13.4 ([-5.7] - [-24]). GLS was correlated with NTproBNP (r=-0.366, p<0.001) and hsTnT (r=-0.5667, p<0.001), and Mayo stage (r=-0.524, p<0.001), but not with clone characteristics such as bone marrow involvement, or free light chain levels. At 6-month landmark, hematologic response rate was 76% for CR/VGPR, 15% PR and 7% NR/PD. Graded cardiac response rates for carCR, carVGPR, carPR and carNR/PD at 6 months were 5.5%, 18%, 35.5% and 50%, at 12 months was 8%, 28%, 35%, 29% and at 24 months 10%, 41%, 28% and 21%, respectively.
Per baseline GLS levels (≥ -9% vs < -9%) graded cardiac response rates at 6 months were 9.8%, 11.5%, 31% and 47.5%, vs 0%, 21%, 39%, 31% and 40% for CR, VGPR, PR, NR/PD (p=0.034), at 12 months was 19%, 14% , 39.5% and 30% vs 4%, 37%, 28% and 31.5% (p=0.012) and at 24 months was 6%, 23%, 29% and 43% vs 7%, 37% 19% and 37% (p=0.508). Baseline GLS was significantly associated with graded cardiac response. In binary logistic regression analysis lower baseline GLS was significantly associated with increased likelihood of achieving carCR at 6 months (OR 1.53, 95% CI 1.139-2.071, p=0.005) and at 12 months (OR 1.345, 95% CI 1.114-1.624, p=0.002). After adjustment for hematologic response at 6 months, baseline GLS remained independent predictor of achieving carCR at 6 months (OR 1.638, 95% CI 1.166-2.303, p=0.004) and at 12 months (OR 1.330, 95% CI 1.107-.599, p=0.002).
Median OS of the whole cohort was 70 months. In univariate analysis GLS < -9% was associated with improved survival (median OS not reached vs 67.7 months; HR 0.561, 95%CI 0.326-0.968, p=0.038). Among patients with GLS >-9, achievement of complete cardiac response at 12 and 24 months was associated with longer OS (Figure 1), while there was no significant difference at 6 months for patients with GLS < -9%. Estimated 2-year survival rate per graded cardiac response at 6 months was 74%, 92% and 100% (p=0.042) for carNR, carPR and carVGPR for patients with impaired GLS (≥-9%) and 73%, 95%, 100% and 100%(p=0.135) for carNR, carPR, carVGPR and carCR.
Summary/Conclusion:
In patients with cardiac AL amyloidosis GLS≥-9% at baseline is associated with lower probability of achieving complete cardiac response independently of hematologic response. However, achievement of carCR is associated with improved outcomes of patients with impaired GLS and this highlights the importance of very effective anti-clonal treatments.
Keyword(s): Free light chain | AL amyloidosis | Plasma cells