Purpura: primary systemic amyloidosis manifestation.
Autor(a) principal: | |
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Data de Publicação: | 2009 |
Outros Autores: | , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1691 |
Resumo: | Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are important in diagnostic suspicion, once they appear in early stages of disease. We report a 71-years-old female patient, with disseminated purpura and cutaneous fragility with 6 months of evolution, accompanied by recent complaints of dysphagy. The first laboratory evaluation didn't show any alterations. The histological and immunohistochemical study of subcutaneous abdominal fat and skin biopsy showed lambda type amyloid protein. In the systemic work-up, we highlight a proteinúria > 1g/24h with Bence Jones proteins and the presence of monoclonal immunoglobulin light chain (lambda type) in serum immunoelectrophoresis. With the diagnosis of primary systemic amyloidosis, treatment with prednisolone and melphalan was started. |
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Purpura: primary systemic amyloidosis manifestation.Púrpura: manifestação de amiloidose sistémica primária.Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are important in diagnostic suspicion, once they appear in early stages of disease. We report a 71-years-old female patient, with disseminated purpura and cutaneous fragility with 6 months of evolution, accompanied by recent complaints of dysphagy. The first laboratory evaluation didn't show any alterations. The histological and immunohistochemical study of subcutaneous abdominal fat and skin biopsy showed lambda type amyloid protein. In the systemic work-up, we highlight a proteinúria > 1g/24h with Bence Jones proteins and the presence of monoclonal immunoglobulin light chain (lambda type) in serum immunoelectrophoresis. With the diagnosis of primary systemic amyloidosis, treatment with prednisolone and melphalan was started.Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are important in diagnostic suspicion, once they appear in early stages of disease. We report a 71-years-old female patient, with disseminated purpura and cutaneous fragility with 6 months of evolution, accompanied by recent complaints of dysphagy. The first laboratory evaluation didn't show any alterations. The histological and immunohistochemical study of subcutaneous abdominal fat and skin biopsy showed lambda type amyloid protein. In the systemic work-up, we highlight a proteinúria > 1g/24h with Bence Jones proteins and the presence of monoclonal immunoglobulin light chain (lambda type) in serum immunoelectrophoresis. With the diagnosis of primary systemic amyloidosis, treatment with prednisolone and melphalan was started.Ordem dos Médicos2009-06-30info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1691oai:ojs.www.actamedicaportuguesa.com:article/1691Acta Médica Portuguesa; Vol. 22 No. 3 (2009): Maio-Junho; 307-12Acta Médica Portuguesa; Vol. 22 N.º 3 (2009): Maio-Junho; 307-121646-07580870-399Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1691https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1691/1271Lestre, SaraGonçalves, AndreiaJoão, AlexandreFerreira, AnaApetato, Margaridainfo:eu-repo/semantics/openAccess2022-12-20T10:58:33Zoai:ojs.www.actamedicaportuguesa.com:article/1691Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:17:19.165590Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Purpura: primary systemic amyloidosis manifestation. Púrpura: manifestação de amiloidose sistémica primária. |
title |
Purpura: primary systemic amyloidosis manifestation. |
spellingShingle |
Purpura: primary systemic amyloidosis manifestation. Lestre, Sara |
title_short |
Purpura: primary systemic amyloidosis manifestation. |
title_full |
Purpura: primary systemic amyloidosis manifestation. |
title_fullStr |
Purpura: primary systemic amyloidosis manifestation. |
title_full_unstemmed |
Purpura: primary systemic amyloidosis manifestation. |
title_sort |
Purpura: primary systemic amyloidosis manifestation. |
author |
Lestre, Sara |
author_facet |
Lestre, Sara Gonçalves, Andreia João, Alexandre Ferreira, Ana Apetato, Margarida |
author_role |
author |
author2 |
Gonçalves, Andreia João, Alexandre Ferreira, Ana Apetato, Margarida |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Lestre, Sara Gonçalves, Andreia João, Alexandre Ferreira, Ana Apetato, Margarida |
description |
Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are important in diagnostic suspicion, once they appear in early stages of disease. We report a 71-years-old female patient, with disseminated purpura and cutaneous fragility with 6 months of evolution, accompanied by recent complaints of dysphagy. The first laboratory evaluation didn't show any alterations. The histological and immunohistochemical study of subcutaneous abdominal fat and skin biopsy showed lambda type amyloid protein. In the systemic work-up, we highlight a proteinúria > 1g/24h with Bence Jones proteins and the presence of monoclonal immunoglobulin light chain (lambda type) in serum immunoelectrophoresis. With the diagnosis of primary systemic amyloidosis, treatment with prednisolone and melphalan was started. |
publishDate |
2009 |
dc.date.none.fl_str_mv |
2009-06-30 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1691 oai:ojs.www.actamedicaportuguesa.com:article/1691 |
url |
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1691 |
identifier_str_mv |
oai:ojs.www.actamedicaportuguesa.com:article/1691 |
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por |
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por |
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1691 https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1691/1271 |
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info:eu-repo/semantics/openAccess |
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openAccess |
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application/pdf |
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Ordem dos Médicos |
publisher.none.fl_str_mv |
Ordem dos Médicos |
dc.source.none.fl_str_mv |
Acta Médica Portuguesa; Vol. 22 No. 3 (2009): Maio-Junho; 307-12 Acta Médica Portuguesa; Vol. 22 N.º 3 (2009): Maio-Junho; 307-12 1646-0758 0870-399X reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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