Search engine for discovering works of Art, research articles, and books related to Art and Culture
ShareThis
Javascript must be enabled to continue!

P71 | DARATUMUMAB IN PATIENTS WITH AL AMYLOIDOSIS: A PORTUGUESE MULTICENTER REAL-WORLD STUDY

View through CrossRef
Introduction. Light-chain (AL) amyloidosis remains a plasma cell dyscrasia with limited therapeutic options and challenging management. Daratumumab (DARA), an anti-CD38 monoclonal antibody, has demonstrated high efficacy in clinical trials, changing the treatment paradigm of AL. However, real-world data are scarce but essential, particularly in frail and unselected patients.Methods. A multicenter retrospective study in Portugal including AL patients treated with daratumumab in first line (1L) or at relapse, assessing baseline characteristics, treatments, responses, and progression-free survival (PFS) and overall survival (OS).Results. A total of 67 patients were included, median age 65 years (39–84), 55% male, 89.6% ECOG PS 1–2. Median time from symptom onset to diagnosis was 5 months (m) (1–25 m), with 41.8% having 1–2 organs involved and 16.6% ≥3 organs, most frequently heart (73%) and kidney (72%). Mayo 2012 stages were I (16.4%), II (26.9%), III (29.9%), and IV (26.9%). Daratumumab was used in 1L in 68.7% and at relapse in 31.3% of patients. In 1L, DARA achieved superior hematologic responses compared with non-DARA regimens: CR 54% vs. 10%, ≥VGPR 91% vs. 43%, and PD 0% vs. 24%. Organ response was also more frequent in the DARA group (kidney 48% vs. 19%; heart 63% vs. 38%). Regarding toxicity, grade 3–4 hematologic events occurred in 9% (DARA) vs. 10% (non-DARA), cardiovascular events in 17% vs. 10%, and infections in 11% vs. 5%. With a median follow-up of 25 months (2–180), 3-year PFS was 80% vs. 40% (p=0.004) in 1L with and without DARA, respectively, and median OS was not reached in either group (OS 80% at 8 years for the whole cohort). In multivariate analysis, ≥VGPR (HR 0.27; p=0.023), ECOG PS 0–1 (HR 0.17; p=0.018), and use of DARA in 1L (HR 2.56; p=0.04) were independently associated with prolonged PFS, while NT-proBNP ≥8500 ng/mL was associated with inferior OS (HR 4.15; p=0.034).Conclusions. This real-world study confirms the efficacy of DARA in AL, with superior hematologic and organ responses in 1L, albeit with higher cardiovascular and infectious toxicity. Depth of response (≥VGPR), ECOG PS 0–1, and first-line use were associated with longer PFS, whereas elevated NT-proBNP was associated with poorer OS. These data support the use of DARA-based combinations as an early first-line standard therapy and highlight the need for, and importance of, real-world studies.
Title: P71 | DARATUMUMAB IN PATIENTS WITH AL AMYLOIDOSIS: A PORTUGUESE MULTICENTER REAL-WORLD STUDY
Description:
Introduction.
Light-chain (AL) amyloidosis remains a plasma cell dyscrasia with limited therapeutic options and challenging management.
Daratumumab (DARA), an anti-CD38 monoclonal antibody, has demonstrated high efficacy in clinical trials, changing the treatment paradigm of AL.
However, real-world data are scarce but essential, particularly in frail and unselected patients.
Methods.
A multicenter retrospective study in Portugal including AL patients treated with daratumumab in first line (1L) or at relapse, assessing baseline characteristics, treatments, responses, and progression-free survival (PFS) and overall survival (OS).
Results.
A total of 67 patients were included, median age 65 years (39–84), 55% male, 89.
6% ECOG PS 1–2.
Median time from symptom onset to diagnosis was 5 months (m) (1–25 m), with 41.
8% having 1–2 organs involved and 16.
6% ≥3 organs, most frequently heart (73%) and kidney (72%).
Mayo 2012 stages were I (16.
4%), II (26.
9%), III (29.
9%), and IV (26.
9%).
Daratumumab was used in 1L in 68.
7% and at relapse in 31.
3% of patients.
In 1L, DARA achieved superior hematologic responses compared with non-DARA regimens: CR 54% vs.
10%, ≥VGPR 91% vs.
43%, and PD 0% vs.
24%.
Organ response was also more frequent in the DARA group (kidney 48% vs.
19%; heart 63% vs.
38%).
Regarding toxicity, grade 3–4 hematologic events occurred in 9% (DARA) vs.
10% (non-DARA), cardiovascular events in 17% vs.
10%, and infections in 11% vs.
5%.
With a median follow-up of 25 months (2–180), 3-year PFS was 80% vs.
40% (p=0.
004) in 1L with and without DARA, respectively, and median OS was not reached in either group (OS 80% at 8 years for the whole cohort).
In multivariate analysis, ≥VGPR (HR 0.
27; p=0.
023), ECOG PS 0–1 (HR 0.
17; p=0.
018), and use of DARA in 1L (HR 2.
56; p=0.
04) were independently associated with prolonged PFS, while NT-proBNP ≥8500 ng/mL was associated with inferior OS (HR 4.
15; p=0.
034).
Conclusions.
This real-world study confirms the efficacy of DARA in AL, with superior hematologic and organ responses in 1L, albeit with higher cardiovascular and infectious toxicity.
Depth of response (≥VGPR), ECOG PS 0–1, and first-line use were associated with longer PFS, whereas elevated NT-proBNP was associated with poorer OS.
These data support the use of DARA-based combinations as an early first-line standard therapy and highlight the need for, and importance of, real-world studies.

Related Results

Daratumumab in Ittp: A Case Series
Daratumumab in Ittp: A Case Series
Background: Immune-mediated thrombotic thrombocytopenia purpura is an idiopathic autoimmune hematological disorder which clinically presents with a transiently or...
Updates on Emerging Therapies in Cardiac Light Chain (AL) Amyloidosis
Updates on Emerging Therapies in Cardiac Light Chain (AL) Amyloidosis
Introduction: Amyloidosis is a disorder where misfolded proteins get deposited in different tissues. The most common of them is immunoglobulin light chain (AL) depos...
Anemia and outcome in cardiac amyloidosis
Anemia and outcome in cardiac amyloidosis
Abstract Background Anemia is a common comorbidity in patients with cardiac amyloidosis, yet its prognostic significance ...
The Safety of Repurposing Daratumumab for Relapsed or Refractory Autoimmune-Antibody-Mediated Hemolytic Anemia
The Safety of Repurposing Daratumumab for Relapsed or Refractory Autoimmune-Antibody-Mediated Hemolytic Anemia
Background Autoimmune Hemolytic Anemia (AIHA) is a disorder in which red blood cells are destroyed by autoantibodies produced by B-cells and plasma cells. The goa...
Daratumumab in untreated newly diagnosed multiple myeloma
Daratumumab in untreated newly diagnosed multiple myeloma
The treatment of multiple myeloma has evolved markedly in the last decade, but mortality remains high, emphasizing the need for more effective therapies. Daratumumab, a fully human...
Comparative Histopathological Characteristics of Duodenal Involvement in Different Types of Amyloidosis
Comparative Histopathological Characteristics of Duodenal Involvement in Different Types of Amyloidosis
Background/Objectives: The duodenum is commonly involved in systemic amyloidosis. This retrospective observational study describes histoanatomical distributions of different types ...
A Pilot Study of Rare Renal Amyloidosis Based on FFPE Proteomics
A Pilot Study of Rare Renal Amyloidosis Based on FFPE Proteomics
Renal amyloidosis typically manifests albuminuria, nephrotic-range proteinuria, and ultimately progresses to end-stage renal failure if diagnosed late. Different types of renal amy...

Back to Top