AL amyloidosis in the Chilean public health system: a pending debt. Multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group

ABSTRACT Background: Immunoglobulin light chain (AL) amyloidosis is a rare and underdiagnosed entity. Aim: To characterize patients with AL amyloidosis in Chilean public health centers. Material and Methods: We conducted a retrospective, multicenter study. Public centers of the Chilean Monoclonal...

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Autores principales: Peña,Camila, González,José Tomás, López-Vidal,Hernán, Donoso,Javiera, Contreras,Carolina, Vergara,Carmen Gloria, Hojas,Ricardo, Soto,Pablo, Correa,Gonzalo, Valjalo,Ricardo, Ríos,Álvaro, Larrondo,Jorge, Álvarez,Jaime, Rojas,Christine
Lenguaje:English
Publicado: Sociedad Médica de Santiago 2019
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872019001001239
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spelling oai:scielo:S0034-988720190010012392020-01-16AL amyloidosis in the Chilean public health system: a pending debt. Multicenter study of the Chilean Monoclonal Gammopathies Cooperative GroupPeña,CamilaGonzález,José TomásLópez-Vidal,HernánDonoso,JavieraContreras,CarolinaVergara,Carmen GloriaHojas,RicardoSoto,PabloCorrea,GonzaloValjalo,RicardoRíos,ÁlvaroLarrondo,JorgeÁlvarez,JaimeRojas,Christine Amyloidosis Bortezomib Multiple Myeloma Paraproteinemias Transplantation ABSTRACT Background: Immunoglobulin light chain (AL) amyloidosis is a rare and underdiagnosed entity. Aim: To characterize patients with AL amyloidosis in Chilean public health centers. Material and Methods: We conducted a retrospective, multicenter study. Public centers of the Chilean Monoclonal Gammopathies Cooperative Group were asked to search for patients with AL amyloidosis in their databases. Epidemiological, clinical and laboratory characteristics were evaluated. Results: Forty-two patients aged 22 to 84 years were found. Twenty four percent had localized AL amyloidosis; 64% had a lambda light chain clone; 47% were associated with multiple myeloma and 9% with non-Hodgkin lymphoma. The most commonly involved organ was the kidney (76%). Serum free light chains were measured in 31% and an echocardiogram was performed in 74% of patients. Seventeen percent of patients received only palliative care, 17% were treated with bortezomib, 21% with thalidomide, and 40% with melphalan. No patient was transplanted. The mean overall survival (OS) of the group was 19 months. The 5-year OS was 28%. Conclusions: It is important to obtain these realistic, national data to initiate strategies to improve early diagnosis and proper management of this disease.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.147 n.10 20192019-10-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872019001001239en10.4067/s0034-98872019001001239
institution Scielo Chile
collection Scielo Chile
language English
topic Amyloidosis
Bortezomib
Multiple Myeloma
Paraproteinemias
Transplantation
spellingShingle Amyloidosis
Bortezomib
Multiple Myeloma
Paraproteinemias
Transplantation
Peña,Camila
González,José Tomás
López-Vidal,Hernán
Donoso,Javiera
Contreras,Carolina
Vergara,Carmen Gloria
Hojas,Ricardo
Soto,Pablo
Correa,Gonzalo
Valjalo,Ricardo
Ríos,Álvaro
Larrondo,Jorge
Álvarez,Jaime
Rojas,Christine
AL amyloidosis in the Chilean public health system: a pending debt. Multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group
description ABSTRACT Background: Immunoglobulin light chain (AL) amyloidosis is a rare and underdiagnosed entity. Aim: To characterize patients with AL amyloidosis in Chilean public health centers. Material and Methods: We conducted a retrospective, multicenter study. Public centers of the Chilean Monoclonal Gammopathies Cooperative Group were asked to search for patients with AL amyloidosis in their databases. Epidemiological, clinical and laboratory characteristics were evaluated. Results: Forty-two patients aged 22 to 84 years were found. Twenty four percent had localized AL amyloidosis; 64% had a lambda light chain clone; 47% were associated with multiple myeloma and 9% with non-Hodgkin lymphoma. The most commonly involved organ was the kidney (76%). Serum free light chains were measured in 31% and an echocardiogram was performed in 74% of patients. Seventeen percent of patients received only palliative care, 17% were treated with bortezomib, 21% with thalidomide, and 40% with melphalan. No patient was transplanted. The mean overall survival (OS) of the group was 19 months. The 5-year OS was 28%. Conclusions: It is important to obtain these realistic, national data to initiate strategies to improve early diagnosis and proper management of this disease.
author Peña,Camila
González,José Tomás
López-Vidal,Hernán
Donoso,Javiera
Contreras,Carolina
Vergara,Carmen Gloria
Hojas,Ricardo
Soto,Pablo
Correa,Gonzalo
Valjalo,Ricardo
Ríos,Álvaro
Larrondo,Jorge
Álvarez,Jaime
Rojas,Christine
author_facet Peña,Camila
González,José Tomás
López-Vidal,Hernán
Donoso,Javiera
Contreras,Carolina
Vergara,Carmen Gloria
Hojas,Ricardo
Soto,Pablo
Correa,Gonzalo
Valjalo,Ricardo
Ríos,Álvaro
Larrondo,Jorge
Álvarez,Jaime
Rojas,Christine
author_sort Peña,Camila
title AL amyloidosis in the Chilean public health system: a pending debt. Multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group
title_short AL amyloidosis in the Chilean public health system: a pending debt. Multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group
title_full AL amyloidosis in the Chilean public health system: a pending debt. Multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group
title_fullStr AL amyloidosis in the Chilean public health system: a pending debt. Multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group
title_full_unstemmed AL amyloidosis in the Chilean public health system: a pending debt. Multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group
title_sort al amyloidosis in the chilean public health system: a pending debt. multicenter study of the chilean monoclonal gammopathies cooperative group
publisher Sociedad Médica de Santiago
publishDate 2019
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872019001001239
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