Clinical characterization and outcomes of a cohort of colombian patients with AL Amyloidosis
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Background: Amyloid light chain (AL) amyloidosis is characterized by amyloid fibril deposition derived from monoclonal immunoglobulin light chains, resulting in multiorgan dysfunction. Limited data exist on the clinical features of AL amyloidosis.
Objective: This study aims to describe the clinical characteristics, treatments, and outcomes in Colombian patients with AL amyloidosis.
Methods: A retrospective descriptive study was conducted at three high-complexity centers in Medellín, Colombia. Adults with AL amyloidosis diagnosed between 2012 and 2022 were included. Clinical, laboratory, histological, treatment, and survival data were analyzed.
Results: The study included 63 patients. Renal involvement was most prevalent (66%), followed by cardiac involvement (61%). Multiorgan involvement occurred in 61% of patients. Amyloid deposition was most commonly detected in renal biopsy (40%). Bortezomib-based therapy was used in 68%, and 23.8% received high-dose chemotherapy with autologous hematopoietic stem cell transplantation (HDCT-ASCT). Hematological response was observed in 95% of patients with available data. Cardiac and renal organ responses were 15% and 14%, respectively. Median overall survival was 45.1 months (95% CI: 22.2-63.8).
In multivariate analysis, cardiac involvement was significantly associated with inferior overall survival (HR 3.27; 95% CI: 1.23-8.73; p=0.018), HDCT-ASCT had a non-significant trend towards improved overall survival (HR 0.25; 95% CI: 0.06-1.09; p=0.065).
Conclusions: In this study of Colombian patients with AL amyloidosis, renal involvement was more frequent than cardiac involvement. Overall survival and multiorgan involvement were consistent with data from other regions of the world. Multivariate analysis identified
cardiac involvement and HDCT-AHCT as possible prognostic factors.
- bortezomib
- lenalidomide
- amyloidosis
- transplantation
- Immunoglobulin Light-chain
- daratumumab
- Amyloidogenic Proteins
- Plasma Cells
- Multiple Myeloma
- Clone Cells
- Hematopoietic Stem Cell Transplantationt response
Jorge Andrés Lacouture Fierro, Hospital San Vicente, Fundación Rionegro, Departamento de Hematología, Rionegro, Colombia.2 Universidad de Antioquia, Sección de Hematología Clínica, Departamento de Medicina interna, Facultad de Medicina, Medellín, Colombia
https://orcid.org/0000-0002-8398-5807
Daniel Andrés Ribero Vargas, 1. Universidad de Antioquia
https://orcid.org/0000-0002-5402-4599
Juanita Sánchez Cano, Hospital Pablo Tobón Uribe; Medellín; Colombia
https://orcid.org/0009-0003-0133-4088
Lina Maria Gaviria Jaramillo, 1. Universidad de Antioquia, Sección de Hematología Clínica, Departamento de Medicina interna, Facultad de Medicina, Medellín, Colombia 2. Hospital San Vicente Fundación Medellín, Departamento de Hematología, Medellín, Colombia
https://orcid.org/0000-0001-6748-6971
Oliver Gerardo Perilla Suarez, Universidad de Antioquia
https://orcid.org/0000-0001-8399-7253
Kenny Mauricio Galvez Cárdenas, Hospital Pablo Tobón Uribe, Departamento de Hematología, Medellín, Colombia.
https://orcid.org/0000-0003-1192-9053
Sigifredo Ospina Ospina, Universidad de Antioquia
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