Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone
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...
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| Format: | Article |
| Language: | English |
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Sociedade Brasileira de Nefrologia
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| Series: | Brazilian Journal of Nephrology |
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| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002016000400450&lng=en&tlng=en |
| _version_ | 1811329699454910464 |
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| author | João Tadeu Damian Souto Filho Jorge Murilo Grillo Monteiro Inêz Barcellos de Andrade |
| author_facet | João Tadeu Damian Souto Filho Jorge Murilo Grillo Monteiro Inêz Barcellos de Andrade |
| author_sort | João Tadeu Damian Souto Filho |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-04-13T15:49:51Z |
| format | Article |
| id | doaj.art-609ac1863d304a17aeeb2177f0341e02 |
| institution | Directory Open Access Journal |
| issn | 2175-8239 |
| language | English |
| last_indexed | 2024-04-13T15:49:51Z |
| publisher | Sociedade Brasileira de Nefrologia |
| record_format | Article |
| series | Brazilian Journal of Nephrology |
| spelling | doaj.art-609ac1863d304a17aeeb2177f0341e022022-12-22T02:40:53ZengSociedade Brasileira de NefrologiaBrazilian Journal of Nephrology2175-823938445045410.5935/0101-2800.20160071S0101-28002016000400450Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasoneJoão Tadeu Damian Souto FilhoJorge Murilo Grillo MonteiroInêz Barcellos de AndradeAbstract 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002016000400450&lng=en&tlng=encadeias leves de imunoglobulinaparaproteínassíndrome nefrótica |
| spellingShingle | João Tadeu Damian Souto Filho Jorge Murilo Grillo Monteiro Inêz Barcellos de Andrade Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone Brazilian Journal of Nephrology cadeias leves de imunoglobulina paraproteínas síndrome nefrótica |
| title | 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_short | 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 |
| topic | cadeias leves de imunoglobulina paraproteínas síndrome nefrótica |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002016000400450&lng=en&tlng=en |
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