Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency

Severe hepatic failure is rarely a cause of death in patients with immunoglobulin light chain (AL) amyloidosis. We herein report a case of AL amyloidosis involving a bleeding tendency due to factor X deficiency and marked hepatic involvement of amyloidosis. The patient died due to severe liver dysfu...

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Autores principales: Guo, Yong-mei, Takahashi, Nagi, Miyabe, Ken, Yoshida, Makoto, Abe, Fumito, Yamashita, Takaya, Nara, Miho, Yoshioka, Tomoko, Ohashi, Kenichi, Goto, Akiteru, Takahashi, Naoto
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2019
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6859402/
https://www.ncbi.nlm.nih.gov/pubmed/31243220
http://dx.doi.org/10.2169/internalmedicine.2864-19
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author Guo, Yong-mei
Takahashi, Nagi
Miyabe, Ken
Yoshida, Makoto
Abe, Fumito
Yamashita, Takaya
Nara, Miho
Yoshioka, Tomoko
Ohashi, Kenichi
Goto, Akiteru
Takahashi, Naoto
author_facet Guo, Yong-mei
Takahashi, Nagi
Miyabe, Ken
Yoshida, Makoto
Abe, Fumito
Yamashita, Takaya
Nara, Miho
Yoshioka, Tomoko
Ohashi, Kenichi
Goto, Akiteru
Takahashi, Naoto
author_sort Guo, Yong-mei
collection PubMed
description Severe hepatic failure is rarely a cause of death in patients with immunoglobulin light chain (AL) amyloidosis. We herein report a case of AL amyloidosis involving a bleeding tendency due to factor X deficiency and marked hepatic involvement of amyloidosis. The patient died due to severe liver dysfunction two weeks after admission. The diagnosis was confirmed histologically by AL-λ amyloidosis, with the liver and spleen as the main lesions, on an autopsy. As treatment-related toxicity is strong in advanced cases, appropriate treatments are required to improve the prognosis of AL amyloidosis with severe liver dysfunction.
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spelling pubmed-68594022019-11-21 Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency Guo, Yong-mei Takahashi, Nagi Miyabe, Ken Yoshida, Makoto Abe, Fumito Yamashita, Takaya Nara, Miho Yoshioka, Tomoko Ohashi, Kenichi Goto, Akiteru Takahashi, Naoto Intern Med Case Report Severe hepatic failure is rarely a cause of death in patients with immunoglobulin light chain (AL) amyloidosis. We herein report a case of AL amyloidosis involving a bleeding tendency due to factor X deficiency and marked hepatic involvement of amyloidosis. The patient died due to severe liver dysfunction two weeks after admission. The diagnosis was confirmed histologically by AL-λ amyloidosis, with the liver and spleen as the main lesions, on an autopsy. As treatment-related toxicity is strong in advanced cases, appropriate treatments are required to improve the prognosis of AL amyloidosis with severe liver dysfunction. The Japanese Society of Internal Medicine 2019-06-27 2019-10-15 /pmc/articles/PMC6859402/ /pubmed/31243220 http://dx.doi.org/10.2169/internalmedicine.2864-19 Text en Copyright © 2019 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Guo, Yong-mei
Takahashi, Nagi
Miyabe, Ken
Yoshida, Makoto
Abe, Fumito
Yamashita, Takaya
Nara, Miho
Yoshioka, Tomoko
Ohashi, Kenichi
Goto, Akiteru
Takahashi, Naoto
Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency
title Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency
title_full Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency
title_fullStr Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency
title_full_unstemmed Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency
title_short Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency
title_sort immunoglobulin light chain amyloidosis with severe liver dysfunction accompanied by factor x deficiency
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6859402/
https://www.ncbi.nlm.nih.gov/pubmed/31243220
http://dx.doi.org/10.2169/internalmedicine.2864-19
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