Clinical characterization and outcomes of a cohort of colombian patients with AL Amyloidosis.

Autor:	Lacouture Fierro JA; Hospital San Vicente Fundación Rionegro , Departamento de Hematología, Rionegro, Colombia.; Universidad de Antioquia, Sección de Hematología Clínica, Departamento de Medicina interna, Facultad de Medicina, Medellín, Colombia., Ribero Vargas DA; Universidad de Antioquia, Facultad de Medicina Departamento de Medicina interna, Medellín, Colombia.; Hospital Alma Mater de Antioquia, Departamento de Medicina Interna, Medellín, Colombia., Sánchez Cano J; Hospital Pablo Tobón Uribe; Medellín; Colombia., Gaviria Jaramillo LM; Universidad de Antioquia, Sección de Hematología Clínica, Departamento de Medicina interna, Facultad de Medicina, Medellín, Colombia.; Hospital San Vicente Fundación Medellín, Departamento de Hematología, Medellín, Colombia., Perilla Suarez OG; Universidad de Antioquia, Sección de Hematología Clínica, Departamento de Medicina interna, Facultad de Medicina, Medellín, Colombia.; Hospital San Vicente Fundación Medellín, Departamento de Hematología, Medellín, Colombia., Galvez Cárdenas KM; Hospital Pablo Tobón Uribe, Departamento de Hematología, Medellín, Colombia., Ospina Ospina S; Universidad de Antioquia, Instituto de Investigaciones Médicas, Facultad de Medicina, Medellín, Colombia.
Jazyk:	angličtina
Zdroj:	Colombia medica (Cali, Colombia) [Colomb Med (Cali)] 2023 Sep 30; Vol. 54 (3), pp. e2025667. Date of Electronic Publication: 2023 Sep 30 (Print Publication: 2023).
DOI:	10.25100/cm.v54i3.5667
Abstrakt:	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. Competing Interests: Conflict of interest: All authors have declared no conflicts of interest. (Copyright © 2023 Colombia Medica.)
Databáze:	MEDLINE
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