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AL Amyloidosis and Multiple Myeloma: A Complex Scenario in Which Cardiac Involvement Remains the Key Prognostic Factor

Monoclonal gammopathies (MGs) are a wide range of diseases that may evolve or progress over time. Comorbidity plays a critical role in this setting. The co-occurrence of two MGs is not a rare event. The evidence on the association of systemic light chain (AL) amyloidosis and multiple myeloma (MM) is...

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Autores principales: Ríos-Tamayo, Rafael, Krsnik, Isabel, Gómez-Bueno, Manuel, Garcia-Pavia, Pablo, Segovia-Cubero, Javier, Huerta, Ana, Salas, Clara, Silvestre, Ramona Ángeles, Sánchez, Amelia, Manso, Marta, Delgado, Laura, Lahuerta, Juan José, Martínez-López, Joaquín, Duarte, Rafael F.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10382070/
https://www.ncbi.nlm.nih.gov/pubmed/37511893
http://dx.doi.org/10.3390/life13071518
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author Ríos-Tamayo, Rafael
Krsnik, Isabel
Gómez-Bueno, Manuel
Garcia-Pavia, Pablo
Segovia-Cubero, Javier
Huerta, Ana
Salas, Clara
Silvestre, Ramona Ángeles
Sánchez, Amelia
Manso, Marta
Delgado, Laura
Lahuerta, Juan José
Martínez-López, Joaquín
Duarte, Rafael F.
author_facet Ríos-Tamayo, Rafael
Krsnik, Isabel
Gómez-Bueno, Manuel
Garcia-Pavia, Pablo
Segovia-Cubero, Javier
Huerta, Ana
Salas, Clara
Silvestre, Ramona Ángeles
Sánchez, Amelia
Manso, Marta
Delgado, Laura
Lahuerta, Juan José
Martínez-López, Joaquín
Duarte, Rafael F.
author_sort Ríos-Tamayo, Rafael
collection PubMed
description Monoclonal gammopathies (MGs) are a wide range of diseases that may evolve or progress over time. Comorbidity plays a critical role in this setting. The co-occurrence of two MGs is not a rare event. The evidence on the association of systemic light chain (AL) amyloidosis and multiple myeloma (MM) is scarce and controversial. Herein we aim to address this topic in a large series of patients of a referral center. All consecutive AL amyloidosis patients treated at our center from January 2005 to April 2023 were prospectively enrolled in a clinical and epidemiological registry. 141 patients diagnosed with AL amyloidosis were included, of which 7 (5%) had localized whereas 134 presented with systemic disease. The heart was the most frequently affected organ (90.3%). 25 patients (18.7%) fulfilled the IMWG diagnostic criteria of MM (AL/MM). Time-dependent association between AL and MM showed that the synchronous pattern is more frequent than the appearance of a second primary malignancy. The diagnostic delay was six months (m). Patients with AL/MM had a poorer median overall survival (OS) than AL-only patients (35.5 m, CI 95% 0–88.9, vs. 52.6 m, CI 95% 16.7–88.5), but this difference was not statistically significant. The prognosis in AL is dominated by the heart involvement, which is massive in this series. In our Cox regression model, only three prognostic variables remain as independent prognostic factors: age, N-terminal pro-brain natriuretic peptide (≥8500 ng/L), and undergoing an autologous stem cell transplant, whereas left ventricular ejection fraction shows a marginal effect. More and large studies focusing on the AL/MM association are needed to uncover the characteristics and prognostic impact of this association.
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spelling pubmed-103820702023-07-29 AL Amyloidosis and Multiple Myeloma: A Complex Scenario in Which Cardiac Involvement Remains the Key Prognostic Factor Ríos-Tamayo, Rafael Krsnik, Isabel Gómez-Bueno, Manuel Garcia-Pavia, Pablo Segovia-Cubero, Javier Huerta, Ana Salas, Clara Silvestre, Ramona Ángeles Sánchez, Amelia Manso, Marta Delgado, Laura Lahuerta, Juan José Martínez-López, Joaquín Duarte, Rafael F. Life (Basel) Article Monoclonal gammopathies (MGs) are a wide range of diseases that may evolve or progress over time. Comorbidity plays a critical role in this setting. The co-occurrence of two MGs is not a rare event. The evidence on the association of systemic light chain (AL) amyloidosis and multiple myeloma (MM) is scarce and controversial. Herein we aim to address this topic in a large series of patients of a referral center. All consecutive AL amyloidosis patients treated at our center from January 2005 to April 2023 were prospectively enrolled in a clinical and epidemiological registry. 141 patients diagnosed with AL amyloidosis were included, of which 7 (5%) had localized whereas 134 presented with systemic disease. The heart was the most frequently affected organ (90.3%). 25 patients (18.7%) fulfilled the IMWG diagnostic criteria of MM (AL/MM). Time-dependent association between AL and MM showed that the synchronous pattern is more frequent than the appearance of a second primary malignancy. The diagnostic delay was six months (m). Patients with AL/MM had a poorer median overall survival (OS) than AL-only patients (35.5 m, CI 95% 0–88.9, vs. 52.6 m, CI 95% 16.7–88.5), but this difference was not statistically significant. The prognosis in AL is dominated by the heart involvement, which is massive in this series. In our Cox regression model, only three prognostic variables remain as independent prognostic factors: age, N-terminal pro-brain natriuretic peptide (≥8500 ng/L), and undergoing an autologous stem cell transplant, whereas left ventricular ejection fraction shows a marginal effect. More and large studies focusing on the AL/MM association are needed to uncover the characteristics and prognostic impact of this association. MDPI 2023-07-06 /pmc/articles/PMC10382070/ /pubmed/37511893 http://dx.doi.org/10.3390/life13071518 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Ríos-Tamayo, Rafael
Krsnik, Isabel
Gómez-Bueno, Manuel
Garcia-Pavia, Pablo
Segovia-Cubero, Javier
Huerta, Ana
Salas, Clara
Silvestre, Ramona Ángeles
Sánchez, Amelia
Manso, Marta
Delgado, Laura
Lahuerta, Juan José
Martínez-López, Joaquín
Duarte, Rafael F.
AL Amyloidosis and Multiple Myeloma: A Complex Scenario in Which Cardiac Involvement Remains the Key Prognostic Factor
title AL Amyloidosis and Multiple Myeloma: A Complex Scenario in Which Cardiac Involvement Remains the Key Prognostic Factor
title_full AL Amyloidosis and Multiple Myeloma: A Complex Scenario in Which Cardiac Involvement Remains the Key Prognostic Factor
title_fullStr AL Amyloidosis and Multiple Myeloma: A Complex Scenario in Which Cardiac Involvement Remains the Key Prognostic Factor
title_full_unstemmed AL Amyloidosis and Multiple Myeloma: A Complex Scenario in Which Cardiac Involvement Remains the Key Prognostic Factor
title_short AL Amyloidosis and Multiple Myeloma: A Complex Scenario in Which Cardiac Involvement Remains the Key Prognostic Factor
title_sort al amyloidosis and multiple myeloma: a complex scenario in which cardiac involvement remains the key prognostic factor
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10382070/
https://www.ncbi.nlm.nih.gov/pubmed/37511893
http://dx.doi.org/10.3390/life13071518
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