A Case of Severe Cholestasis due to Hepatic AL Amyloidosis
Autor(a) principal: | |
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Data de Publicação: | 2019 |
Outros Autores: | , , , , |
Tipo de documento: | Relatório |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000600007 |
Resumo: | Introduction: Immunoglobulin light chain-associated amyloidosis results from extracellular tissue deposition of fibrilforming monoclonal immunoglobulin light chains, secreted by a clone of plasma cells. Although the liver is often involved histologically, most cases are clinically asymptomatic, and severe intrahepatic cholestasis as the primary manifestation of the disease is rare. Clinical Case: We report an unusual case of primary amyloidosis in a 71-year-old man, presenting with rapidly progressive cholestasis, associated with hepatomegaly. There are a few reported cases in the literature of cholestasis and acute liver failure as the first manifestations of AL amyloidosis. Conclusion: Infiltrative diseases, such as amyloidosis, must be considered when a patient presents with cholestatic hepatitis. A liver biopsy is essential for the diagnosis of amyloidosis when liver test abnormalities dominate the initial clinical presentation. Liver involvement in patients with amyloidosis is often an indicator of poor prognosis. |
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A Case of Severe Cholestasis due to Hepatic AL AmyloidosisCholestasisAmyloidosisLiverMyelomaIntroduction: Immunoglobulin light chain-associated amyloidosis results from extracellular tissue deposition of fibrilforming monoclonal immunoglobulin light chains, secreted by a clone of plasma cells. Although the liver is often involved histologically, most cases are clinically asymptomatic, and severe intrahepatic cholestasis as the primary manifestation of the disease is rare. Clinical Case: We report an unusual case of primary amyloidosis in a 71-year-old man, presenting with rapidly progressive cholestasis, associated with hepatomegaly. There are a few reported cases in the literature of cholestasis and acute liver failure as the first manifestations of AL amyloidosis. Conclusion: Infiltrative diseases, such as amyloidosis, must be considered when a patient presents with cholestatic hepatitis. A liver biopsy is essential for the diagnosis of amyloidosis when liver test abnormalities dominate the initial clinical presentation. Liver involvement in patients with amyloidosis is often an indicator of poor prognosis.Sociedade Portuguesa de Gastrenterologia2019-12-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000600007GE-Portuguese Journal of Gastroenterology v.26 n.6 2019reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000600007Dias,TeresaFerreira,DianaMoreira,HélderNascimento,TelmaSantos,ArsénioCarvalho,Armandoinfo:eu-repo/semantics/openAccess2024-02-06T17:34:00Zoai:scielo:S2341-45452019000600007Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:36:09.528011Repositório 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 |
A Case of Severe Cholestasis due to Hepatic AL Amyloidosis |
title |
A Case of Severe Cholestasis due to Hepatic AL Amyloidosis |
spellingShingle |
A Case of Severe Cholestasis due to Hepatic AL Amyloidosis Dias,Teresa Cholestasis Amyloidosis Liver Myeloma |
title_short |
A Case of Severe Cholestasis due to Hepatic AL Amyloidosis |
title_full |
A Case of Severe Cholestasis due to Hepatic AL Amyloidosis |
title_fullStr |
A Case of Severe Cholestasis due to Hepatic AL Amyloidosis |
title_full_unstemmed |
A Case of Severe Cholestasis due to Hepatic AL Amyloidosis |
title_sort |
A Case of Severe Cholestasis due to Hepatic AL Amyloidosis |
author |
Dias,Teresa |
author_facet |
Dias,Teresa Ferreira,Diana Moreira,Hélder Nascimento,Telma Santos,Arsénio Carvalho,Armando |
author_role |
author |
author2 |
Ferreira,Diana Moreira,Hélder Nascimento,Telma Santos,Arsénio Carvalho,Armando |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Dias,Teresa Ferreira,Diana Moreira,Hélder Nascimento,Telma Santos,Arsénio Carvalho,Armando |
dc.subject.por.fl_str_mv |
Cholestasis Amyloidosis Liver Myeloma |
topic |
Cholestasis Amyloidosis Liver Myeloma |
description |
Introduction: Immunoglobulin light chain-associated amyloidosis results from extracellular tissue deposition of fibrilforming monoclonal immunoglobulin light chains, secreted by a clone of plasma cells. Although the liver is often involved histologically, most cases are clinically asymptomatic, and severe intrahepatic cholestasis as the primary manifestation of the disease is rare. Clinical Case: We report an unusual case of primary amyloidosis in a 71-year-old man, presenting with rapidly progressive cholestasis, associated with hepatomegaly. There are a few reported cases in the literature of cholestasis and acute liver failure as the first manifestations of AL amyloidosis. Conclusion: Infiltrative diseases, such as amyloidosis, must be considered when a patient presents with cholestatic hepatitis. A liver biopsy is essential for the diagnosis of amyloidosis when liver test abnormalities dominate the initial clinical presentation. Liver involvement in patients with amyloidosis is often an indicator of poor prognosis. |
publishDate |
2019 |
dc.date.none.fl_str_mv |
2019-12-01 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
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info:eu-repo/semantics/report |
format |
report |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000600007 |
url |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000600007 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000600007 |
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 Portuguesa de Gastrenterologia |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Gastrenterologia |
dc.source.none.fl_str_mv |
GE-Portuguese Journal of Gastroenterology v.26 n.6 2019 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 |
instacron_str |
RCAAP |
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RCAAP |
reponame_str |
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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1799137413652545536 |