Purpura: primary systemic amyloidosis manifestation.

Detalhes bibliográficos
Autor(a) principal: Lestre, Sara
Data de Publicação: 2009
Outros Autores: Gonçalves, Andreia, João, Alexandre, Ferreira, Ana, Apetato, Margarida
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.
id RCAP_81e96b68934c8d2389ac4ac186c35c32
oai_identifier_str oai:ojs.www.actamedicaportuguesa.com:article/1691
network_acronym_str RCAP
network_name_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository_id_str 7160
spelling 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
dc.language.iso.fl_str_mv por
language por
dc.relation.none.fl_str_mv https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1691
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1691/1271
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv 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
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
repository.mail.fl_str_mv
_version_ 1799130626212757504

Registros relacionados