A Case of Severe Cholestasis due to Hepatic AL Amyloidosis

Detalhes bibliográficos
Autor(a) principal: Dias, Teresa
Data de Publicação: 2019
Outros Autores: Ferreira, Diana, Moreira, Hélder, Nascimento, Telma, Santos, Arsénio, Carvalho, Armando
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10316/106999
https://doi.org/10.1159/000496185
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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spelling A Case of Severe Cholestasis due to Hepatic AL AmyloidosisUm caso de colestase grave por amiloidose AL com envolvimento hepáticoCholestasisAmyloidosisLiverMyelomaColestaseAmiloidoseFígadoMielomaIntroduction: 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.Introdução: A amiloidose associada a cadeias leves resulta da deposição tecidular de cadeias leves de imunoglobulina monoclonal, segregada por um clone de células plasmáticas. Embora o fígado esteja com frequência envolvido histologicamente, a maioria dos casos é clinicamente assintomática, e a colestase intra-hepática grave como manifestação primária da doença é rara. Caso clínico: Os autores relatam um caso raro de amiloidose primária num homem de 71 anos, manifestando colestase rapidamente progressiva, associada a hepatomegalia. Existem poucos casos descritos na literatura de colestase e insuficiência hepática aguda como a primeira manifestação de amiloidose AL. Conclusão: As doenças infiltrativas, como a amiloidose, devem ser consideradas quando um doente apresenta hepatite colestática. A biópsia hepática é essencial para o diagnóstico de amiloidose quando as alterações das provas hepáticas dominam a apresentação inicial. O envolvimento do fígado em pacientes com amiloidose é com frequência um indicador de mau prognóstico.Karger2019-10info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttp://hdl.handle.net/10316/106999http://hdl.handle.net/10316/106999https://doi.org/10.1159/000496185eng2341-4545Dias, TeresaFerreira, DianaMoreira, HélderNascimento, TelmaSantos, ArsénioCarvalho, Armandoinfo:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-05-08T11:57:54Zoai:estudogeral.uc.pt:10316/106999Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T21:23:22.866197Repositório 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
Um caso de colestase grave por amiloidose AL com envolvimento hepático
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
Colestase
Amiloidose
Fígado
Mieloma
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
Colestase
Amiloidose
Fígado
Mieloma
topic Cholestasis
Amyloidosis
Liver
Myeloma
Colestase
Amiloidose
Fígado
Mieloma
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 2019-10
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 http://hdl.handle.net/10316/106999
http://hdl.handle.net/10316/106999
https://doi.org/10.1159/000496185
url http://hdl.handle.net/10316/106999
https://doi.org/10.1159/000496185
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 2341-4545
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
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dc.publisher.none.fl_str_mv Karger
publisher.none.fl_str_mv Karger
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instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
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instacron_str RCAAP
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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
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