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Outcome of patients with systemic light chain amyloidosis with concurrent renal and cardiac involvement
Talha Badar1, Amanda Megan Cornelison1, Nina D Shah1
1Department of Stem Cell Transplantation and Cellular Therapy, University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Insights
Patients with systemic light chain amyloidosis (AL) and both cardiac and renal involvement had outcomes comparable to those with cardiac AL alone, but worse than those with renal AL alone after treatment. This highlights the prognostic impact of combined organ involvement.
Area of Science:
- Hematology
- Oncology
- Nephrology
- Cardiology
Background:
- Systemic light chain amyloidosis (AL) commonly affects the heart, leading to worse outcomes, particularly with multi-organ involvement.
- The prognostic significance of concurrent cardiac and renal involvement in AL amyloidosis requires further elucidation.
Purpose of the Study:
- To compare outcomes in patients with AL amyloidosis and isolated renal, isolated cardiac, or concurrent cardiac and renal involvement.
Main Methods:
- Retrospective analysis of 129 AL amyloidosis patients treated with high-dose chemotherapy and autologous hematopoietic stem cell transplantation (auto-HCT) between 1997 and 2014.
- Patients were categorized into three groups: renal involvement only (n=62), cardiac involvement only (n=20), and both cardiac and renal involvement (n=17).
Main Results:
- Hematological response rates were similar across all groups (69-82%).
- Organ response rates were higher in patients with combined cardiac and renal involvement (70%) compared to isolated renal (39%) or cardiac (42%) involvement.
- Median progression-free survival (PFS) was significantly longer in patients with combined cardiac and renal involvement (21 months) and cardiac involvement (13.3 months) compared to renal involvement (not reached) (P=0.02).
- Median overall survival (OS) was 120 months for renal, 46 months for cardiac, and 60 months for combined cardiac and renal involvement (P=0.1).
Conclusions:
- Concurrent cardiac and renal AL amyloidosis demonstrates a comparable PFS and OS to cardiac AL amyloidosis alone, but worse outcomes than renal AL amyloidosis alone.
- Combined cardiac and renal involvement is associated with a higher organ response rate post-auto-HCT.
Abstract:
Cardiac involvement in systemic light chain amyloidosis (AL) is generally associated with a worse outcome, especially if other organs are also involved. We sought to determine whether concurrent cardiac and renal involvement were associated with a worse outcome than either organ alone. We identified 129 patients with AL, who received high-dose chemotherapy followed by autologous hematopoietic stem cell transplantation (auto-HCT) at our institution between 1997 and 2014. Ninety-nine patients had either renal (group 1: n = 62, 62%), cardiac (group 2: n = 20, 20%), or both cardiac and renal (group 3: n = 17, 17%) involvement. The overall hematological response rate (CR+VGPR+PR) post-auto-HCT in groups 1, 2, and 3 was 69%, 74% and 82%, respectively (P = 0.62). Overall, organ response in groups 1, 2, and 3 was 39%, 42%, and 70%, respectively. The median PFS from auto-HCT in groups 1, 2, and 3 was not reached (NR), 13.3 and 21 months, respectively (P = 0.02). The median OS in groups 1, 2, and 3 was 120, 46, and 60 months, respectively (P = 0.1). In conclusion, median PFS and OS in patients with concurrent cardiac and renal AL were comparable to patients with cardiac AL only, but worse than patients with renal AL.
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