Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone
Autor(a) principal: | |
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Data de Publicação: | 2016 |
Outros Autores: | , |
Tipo de documento: | Relatório |
Idioma: | eng |
Título da fonte: | Jornal Brasileiro de Nefrologia |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002016000400450 |
Resumo: | Abstract Light chain deposition disease (LCDD) is a rare clinical entity characterized by the deposition of light chain immunoglobulins in different tissues and primarily affects the kidneys, followed by the liver and heart. This disease often manifests as nephrotic syndrome with marked proteinuria and rapid deterioration of renal function. More than 50% of cases are secondary to multiple myeloma or other lymphoproliferative diseases, with a well-established treatment aimed at controlling the underlying disease. In rare cases, there is no detection of an associated hematological disease, referred to as idiopathic LCDD. In these cases, there is no evidence-based consensus on the therapeutic approach, and management is based on the clinical experience of reported cases. Here we report a case of idiopathic LCDD treated with bortezomib and dexamethasone with complete hematologic responses, significant reduction of proteinuria, and improved renal function. |
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Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasoneimmunoglobulin light chainsnephrotic syndromeparaproteinsAbstract Light chain deposition disease (LCDD) is a rare clinical entity characterized by the deposition of light chain immunoglobulins in different tissues and primarily affects the kidneys, followed by the liver and heart. This disease often manifests as nephrotic syndrome with marked proteinuria and rapid deterioration of renal function. More than 50% of cases are secondary to multiple myeloma or other lymphoproliferative diseases, with a well-established treatment aimed at controlling the underlying disease. In rare cases, there is no detection of an associated hematological disease, referred to as idiopathic LCDD. In these cases, there is no evidence-based consensus on the therapeutic approach, and management is based on the clinical experience of reported cases. Here we report a case of idiopathic LCDD treated with bortezomib and dexamethasone with complete hematologic responses, significant reduction of proteinuria, and improved renal function.Sociedade Brasileira de Nefrologia2016-12-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002016000400450Brazilian Journal of Nephrology v.38 n.4 2016reponame:Jornal Brasileiro de Nefrologiainstname:Sociedade Brasileira de Nefrologia (SBN)instacron:SBN10.5935/0101-2800.20160071info:eu-repo/semantics/openAccessSouto Filho,João Tadeu DamianMonteiro,Jorge Murilo GrilloAndrade,Inêz Barcellos deeng2016-12-19T00:00:00Zoai:scielo:S0101-28002016000400450Revistahttp://www.bjn.org.br/ONGhttps://old.scielo.br/oai/scielo-oai.php||jbn@sbn.org.br2175-82390101-2800opendoar:2016-12-19T00:00Jornal Brasileiro de Nefrologia - Sociedade Brasileira de Nefrologia (SBN)false |
dc.title.none.fl_str_mv |
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone |
title |
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone |
spellingShingle |
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone Souto Filho,João Tadeu Damian immunoglobulin light chains nephrotic syndrome paraproteins |
title_short |
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone |
title_full |
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone |
title_fullStr |
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone |
title_full_unstemmed |
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone |
title_sort |
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone |
author |
Souto Filho,João Tadeu Damian |
author_facet |
Souto Filho,João Tadeu Damian Monteiro,Jorge Murilo Grillo Andrade,Inêz Barcellos de |
author_role |
author |
author2 |
Monteiro,Jorge Murilo Grillo Andrade,Inêz Barcellos de |
author2_role |
author author |
dc.contributor.author.fl_str_mv |
Souto Filho,João Tadeu Damian Monteiro,Jorge Murilo Grillo Andrade,Inêz Barcellos de |
dc.subject.por.fl_str_mv |
immunoglobulin light chains nephrotic syndrome paraproteins |
topic |
immunoglobulin light chains nephrotic syndrome paraproteins |
description |
Abstract Light chain deposition disease (LCDD) is a rare clinical entity characterized by the deposition of light chain immunoglobulins in different tissues and primarily affects the kidneys, followed by the liver and heart. This disease often manifests as nephrotic syndrome with marked proteinuria and rapid deterioration of renal function. More than 50% of cases are secondary to multiple myeloma or other lymphoproliferative diseases, with a well-established treatment aimed at controlling the underlying disease. In rare cases, there is no detection of an associated hematological disease, referred to as idiopathic LCDD. In these cases, there is no evidence-based consensus on the therapeutic approach, and management is based on the clinical experience of reported cases. Here we report a case of idiopathic LCDD treated with bortezomib and dexamethasone with complete hematologic responses, significant reduction of proteinuria, and improved renal function. |
publishDate |
2016 |
dc.date.none.fl_str_mv |
2016-12-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/report |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
report |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002016000400450 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002016000400450 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.5935/0101-2800.20160071 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Sociedade Brasileira de Nefrologia |
publisher.none.fl_str_mv |
Sociedade Brasileira de Nefrologia |
dc.source.none.fl_str_mv |
Brazilian Journal of Nephrology v.38 n.4 2016 reponame:Jornal Brasileiro de Nefrologia instname:Sociedade Brasileira de Nefrologia (SBN) instacron:SBN |
instname_str |
Sociedade Brasileira de Nefrologia (SBN) |
instacron_str |
SBN |
institution |
SBN |
reponame_str |
Jornal Brasileiro de Nefrologia |
collection |
Jornal Brasileiro de Nefrologia |
repository.name.fl_str_mv |
Jornal Brasileiro de Nefrologia - Sociedade Brasileira de Nefrologia (SBN) |
repository.mail.fl_str_mv |
||jbn@sbn.org.br |
_version_ |
1752122064214425600 |