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: 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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spelling 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
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dc.language.iso.fl_str_mv eng
language eng
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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)
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