Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina

Autores
Posadas Martinez, Maria Lourdes; Aguirre, Maria Adela; Brulc, Erika B.; Saez, Maria Soledad; Sorroche, Patricia Beatriz; Machnicki, Gerardo; Fernandez, Mariana; Nucifora, Elsa Mercedes
Año de publicación
2022
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Light chain (AL) amyloidosis is a form of systemic amyloidosis, causing organ dysfunction, mainly affecting the heart and kidney. Patient-tailored and risk-adapted decision making is critical in AL amyloidosis management. There is limited real-world evidence data from Argentina and Latin America regarding the treatment approaches for AL amyloidosis. This retrospective cohort study aimed to describe the treatment patterns and outcomes in adult patients (>18 years) diagnosed with AL amyloidosis at the Hospital Italiano in Buenos Aires, Argentina, using a 10-yearfollow-up data (June 1, 2010 to May 31, 2019) from the institutional registry of amyloidosis (IRA). The study population had a mean age of 63 years and 54.4% weremale. Heart and kidney were the most frequently affected organs. Of the 90 eligible patients included in the study, 70underwent treatment. Bortezomib-based regimen was the preferred first-line treatment (75.7% patients). Overall, 54.4% of the patients presented a deep response (complete or very good partial response). Median overall survival (OS) was 5years, the 1-year OS and progression free survival rates were 80% (95% confidence interval [CI]: 68–87) and 80% (95%CI 68–87)), respectively. This study provides vital real-world evidence for the long-term treatment patterns and survival in a large cohort of AL amyloidosis patients in Argentina.
Fil: Posadas Martinez, Maria Lourdes. Hospital Italiano; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Aguirre, Maria Adela. Consejo Nacional de Investigaciones Cientificas y Tecnicas. Oficina de Coordinacion Administrativa Houssay. Instituto de Medicina Traslacional E Ingenieria Biomedica. - Hospital Italiano. Instituto de Medicina Traslacional E Ingenieria Biomedica. - Instituto Universitario Hospital Italiano de Buenos Aires. Instituto de Medicina Traslacional E Ingenieria Biomedica.; Argentina
Fil: Brulc, Erika B.. Hospital Italiano; Argentina
Fil: Saez, Maria Soledad. Hospital Italiano; Argentina
Fil: Sorroche, Patricia Beatriz. Hospital Italiano; Argentina
Fil: Machnicki, Gerardo. No especifíca;
Fil: Fernandez, Mariana. No especifíca;
Fil: Nucifora, Elsa Mercedes. Hospital Italiano; Argentina
Materia
LIGHT CHAIN AMYLOIDOSIS
TREATMENT PATTERNS
AMYLOIDOSIS ARGENTINA
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/217849
Acceder
id CONICETDig_bb8fb42c1d645cf40d893e75723329c9
oai_identifier_str oai:ri.conicet.gov.ar:11336/217849
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in ArgentinaPosadas Martinez, Maria LourdesAguirre, Maria AdelaBrulc, Erika B.Saez, Maria SoledadSorroche, Patricia BeatrizMachnicki, GerardoFernandez, MarianaNucifora, Elsa MercedesLIGHT CHAIN AMYLOIDOSISTREATMENT PATTERNSAMYLOIDOSIS ARGENTINAhttps://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3https://purl.org/becyt/ford/3.3https://purl.org/becyt/ford/3Light chain (AL) amyloidosis is a form of systemic amyloidosis, causing organ dysfunction, mainly affecting the heart and kidney. Patient-tailored and risk-adapted decision making is critical in AL amyloidosis management. There is limited real-world evidence data from Argentina and Latin America regarding the treatment approaches for AL amyloidosis. This retrospective cohort study aimed to describe the treatment patterns and outcomes in adult patients (>18 years) diagnosed with AL amyloidosis at the Hospital Italiano in Buenos Aires, Argentina, using a 10-yearfollow-up data (June 1, 2010 to May 31, 2019) from the institutional registry of amyloidosis (IRA). The study population had a mean age of 63 years and 54.4% weremale. Heart and kidney were the most frequently affected organs. Of the 90 eligible patients included in the study, 70underwent treatment. Bortezomib-based regimen was the preferred first-line treatment (75.7% patients). Overall, 54.4% of the patients presented a deep response (complete or very good partial response). Median overall survival (OS) was 5years, the 1-year OS and progression free survival rates were 80% (95% confidence interval [CI]: 68–87) and 80% (95%CI 68–87)), respectively. This study provides vital real-world evidence for the long-term treatment patterns and survival in a large cohort of AL amyloidosis patients in Argentina.Fil: Posadas Martinez, Maria Lourdes. Hospital Italiano; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Aguirre, Maria Adela. Consejo Nacional de Investigaciones Cientificas y Tecnicas. Oficina de Coordinacion Administrativa Houssay. Instituto de Medicina Traslacional E Ingenieria Biomedica. - Hospital Italiano. Instituto de Medicina Traslacional E Ingenieria Biomedica. - Instituto Universitario Hospital Italiano de Buenos Aires. Instituto de Medicina Traslacional E Ingenieria Biomedica.; ArgentinaFil: Brulc, Erika B.. Hospital Italiano; ArgentinaFil: Saez, Maria Soledad. Hospital Italiano; ArgentinaFil: Sorroche, Patricia Beatriz. Hospital Italiano; ArgentinaFil: Machnicki, Gerardo. No especifíca;Fil: Fernandez, Mariana. No especifíca;Fil: Nucifora, Elsa Mercedes. Hospital Italiano; ArgentinaPublic Library of Science2022-10info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/217849Posadas Martinez, Maria Lourdes; Aguirre, Maria Adela; Brulc, Erika B.; Saez, Maria Soledad; Sorroche, Patricia Beatriz; et al.; Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina; Public Library of Science; Plos One; 17; 10-2022; 1-141932-6203CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/doi/10.1371/journal.pone.0274578info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-03T09:54:46Zoai:ri.conicet.gov.ar:11336/217849instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-03 09:54:46.751CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina
title Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina
spellingShingle Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina
Posadas Martinez, Maria Lourdes
LIGHT CHAIN AMYLOIDOSIS
TREATMENT PATTERNS
AMYLOIDOSIS ARGENTINA
title_short Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina
title_full Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina
title_fullStr Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina
title_full_unstemmed Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina
title_sort Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina
dc.creator.none.fl_str_mv Posadas Martinez, Maria Lourdes
Aguirre, Maria Adela
Brulc, Erika B.
Saez, Maria Soledad
Sorroche, Patricia Beatriz
Machnicki, Gerardo
Fernandez, Mariana
Nucifora, Elsa Mercedes
author Posadas Martinez, Maria Lourdes
author_facet Posadas Martinez, Maria Lourdes
Aguirre, Maria Adela
Brulc, Erika B.
Saez, Maria Soledad
Sorroche, Patricia Beatriz
Machnicki, Gerardo
Fernandez, Mariana
Nucifora, Elsa Mercedes
author_role author
author2 Aguirre, Maria Adela
Brulc, Erika B.
Saez, Maria Soledad
Sorroche, Patricia Beatriz
Machnicki, Gerardo
Fernandez, Mariana
Nucifora, Elsa Mercedes
author2_role author
author
author
author
author
author
author
dc.subject.none.fl_str_mv LIGHT CHAIN AMYLOIDOSIS
TREATMENT PATTERNS
AMYLOIDOSIS ARGENTINA
topic LIGHT CHAIN AMYLOIDOSIS
TREATMENT PATTERNS
AMYLOIDOSIS ARGENTINA
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
https://purl.org/becyt/ford/3.3
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Light chain (AL) amyloidosis is a form of systemic amyloidosis, causing organ dysfunction, mainly affecting the heart and kidney. Patient-tailored and risk-adapted decision making is critical in AL amyloidosis management. There is limited real-world evidence data from Argentina and Latin America regarding the treatment approaches for AL amyloidosis. This retrospective cohort study aimed to describe the treatment patterns and outcomes in adult patients (>18 years) diagnosed with AL amyloidosis at the Hospital Italiano in Buenos Aires, Argentina, using a 10-yearfollow-up data (June 1, 2010 to May 31, 2019) from the institutional registry of amyloidosis (IRA). The study population had a mean age of 63 years and 54.4% weremale. Heart and kidney were the most frequently affected organs. Of the 90 eligible patients included in the study, 70underwent treatment. Bortezomib-based regimen was the preferred first-line treatment (75.7% patients). Overall, 54.4% of the patients presented a deep response (complete or very good partial response). Median overall survival (OS) was 5years, the 1-year OS and progression free survival rates were 80% (95% confidence interval [CI]: 68–87) and 80% (95%CI 68–87)), respectively. This study provides vital real-world evidence for the long-term treatment patterns and survival in a large cohort of AL amyloidosis patients in Argentina.
Fil: Posadas Martinez, Maria Lourdes. Hospital Italiano; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Aguirre, Maria Adela. Consejo Nacional de Investigaciones Cientificas y Tecnicas. Oficina de Coordinacion Administrativa Houssay. Instituto de Medicina Traslacional E Ingenieria Biomedica. - Hospital Italiano. Instituto de Medicina Traslacional E Ingenieria Biomedica. - Instituto Universitario Hospital Italiano de Buenos Aires. Instituto de Medicina Traslacional E Ingenieria Biomedica.; Argentina
Fil: Brulc, Erika B.. Hospital Italiano; Argentina
Fil: Saez, Maria Soledad. Hospital Italiano; Argentina
Fil: Sorroche, Patricia Beatriz. Hospital Italiano; Argentina
Fil: Machnicki, Gerardo. No especifíca;
Fil: Fernandez, Mariana. No especifíca;
Fil: Nucifora, Elsa Mercedes. Hospital Italiano; Argentina
description Light chain (AL) amyloidosis is a form of systemic amyloidosis, causing organ dysfunction, mainly affecting the heart and kidney. Patient-tailored and risk-adapted decision making is critical in AL amyloidosis management. There is limited real-world evidence data from Argentina and Latin America regarding the treatment approaches for AL amyloidosis. This retrospective cohort study aimed to describe the treatment patterns and outcomes in adult patients (>18 years) diagnosed with AL amyloidosis at the Hospital Italiano in Buenos Aires, Argentina, using a 10-yearfollow-up data (June 1, 2010 to May 31, 2019) from the institutional registry of amyloidosis (IRA). The study population had a mean age of 63 years and 54.4% weremale. Heart and kidney were the most frequently affected organs. Of the 90 eligible patients included in the study, 70underwent treatment. Bortezomib-based regimen was the preferred first-line treatment (75.7% patients). Overall, 54.4% of the patients presented a deep response (complete or very good partial response). Median overall survival (OS) was 5years, the 1-year OS and progression free survival rates were 80% (95% confidence interval [CI]: 68–87) and 80% (95%CI 68–87)), respectively. This study provides vital real-world evidence for the long-term treatment patterns and survival in a large cohort of AL amyloidosis patients in Argentina.
publishDate 2022
dc.date.none.fl_str_mv 2022-10
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/217849
Posadas Martinez, Maria Lourdes; Aguirre, Maria Adela; Brulc, Erika B.; Saez, Maria Soledad; Sorroche, Patricia Beatriz; et al.; Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina; Public Library of Science; Plos One; 17; 10-2022; 1-14
1932-6203
CONICET Digital
CONICET
url http://hdl.handle.net/11336/217849
identifier_str_mv Posadas Martinez, Maria Lourdes; Aguirre, Maria Adela; Brulc, Erika B.; Saez, Maria Soledad; Sorroche, Patricia Beatriz; et al.; Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina; Public Library of Science; Plos One; 17; 10-2022; 1-14
1932-6203
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/doi/10.1371/journal.pone.0274578
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
application/pdf
application/pdf
dc.publisher.none.fl_str_mv Public Library of Science
publisher.none.fl_str_mv Public Library of Science
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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score 13.13397

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