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Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report

BACKGROUND: AL Amyloidosis is known to be a systemic disease affecting multiple organs and tissue while it’s rare that patients present with gastrointestinal symptoms at first and later develop multiple-organ dysfuction. Clinical signs are not specific and the diagnosis is rarely given before perfor...

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Autores principales: Tian, Li, Tang, Anliu, Zhang, Xian, Mei, Zhen, Liu, Fen, Li, Jingbo, Li, Xiayu, Ai, Feiyan, Wang, Xiaoyan, Shen, Shourong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5460434/
https://www.ncbi.nlm.nih.gov/pubmed/28583087
http://dx.doi.org/10.1186/s12876-017-0628-3
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author Tian, Li
Tang, Anliu
Zhang, Xian
Mei, Zhen
Liu, Fen
Li, Jingbo
Li, Xiayu
Ai, Feiyan
Wang, Xiaoyan
Shen, Shourong
author_facet Tian, Li
Tang, Anliu
Zhang, Xian
Mei, Zhen
Liu, Fen
Li, Jingbo
Li, Xiayu
Ai, Feiyan
Wang, Xiaoyan
Shen, Shourong
author_sort Tian, Li
collection PubMed
description BACKGROUND: AL Amyloidosis is known to be a systemic disease affecting multiple organs and tissue while it’s rare that patients present with gastrointestinal symptoms at first and later develop multiple-organ dysfuction. Clinical signs are not specific and the diagnosis is rarely given before performing immunofixation and endoscopy with multiple biopsies. We would like to emphasize the value of precise diagnostic process of AL amyloidosis. CASE PRESENTATION: In this case report, we describe a 56-year-old man who presented with recurrent periumbilical pain for 4 months and gradually worsened over a month. After a series of tests, he was finally diagnosed with primary systemic AL amyloidosis. He was treated with a chemotherapy regimen (Melphalan, dexamethasone and thalidomide) achieving a good clinical response. CONCLUSION: On account of the high misdiagnosis rate, establishing the most precise diagnosis in first time with typing amyloidogenic protein becomes increasingly vital. Although the presenting feature is usually nonspecific, AL amyloidosis ought to be considered when multiple organs are involved in a short period.
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spelling pubmed-54604342017-06-07 Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report Tian, Li Tang, Anliu Zhang, Xian Mei, Zhen Liu, Fen Li, Jingbo Li, Xiayu Ai, Feiyan Wang, Xiaoyan Shen, Shourong BMC Gastroenterol Case Report BACKGROUND: AL Amyloidosis is known to be a systemic disease affecting multiple organs and tissue while it’s rare that patients present with gastrointestinal symptoms at first and later develop multiple-organ dysfuction. Clinical signs are not specific and the diagnosis is rarely given before performing immunofixation and endoscopy with multiple biopsies. We would like to emphasize the value of precise diagnostic process of AL amyloidosis. CASE PRESENTATION: In this case report, we describe a 56-year-old man who presented with recurrent periumbilical pain for 4 months and gradually worsened over a month. After a series of tests, he was finally diagnosed with primary systemic AL amyloidosis. He was treated with a chemotherapy regimen (Melphalan, dexamethasone and thalidomide) achieving a good clinical response. CONCLUSION: On account of the high misdiagnosis rate, establishing the most precise diagnosis in first time with typing amyloidogenic protein becomes increasingly vital. Although the presenting feature is usually nonspecific, AL amyloidosis ought to be considered when multiple organs are involved in a short period. BioMed Central 2017-06-05 /pmc/articles/PMC5460434/ /pubmed/28583087 http://dx.doi.org/10.1186/s12876-017-0628-3 Text en © The Author(s). 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Tian, Li
Tang, Anliu
Zhang, Xian
Mei, Zhen
Liu, Fen
Li, Jingbo
Li, Xiayu
Ai, Feiyan
Wang, Xiaoyan
Shen, Shourong
Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report
title Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report
title_full Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report
title_fullStr Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report
title_full_unstemmed Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report
title_short Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report
title_sort incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic al amyloidosis: a rare case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5460434/
https://www.ncbi.nlm.nih.gov/pubmed/28583087
http://dx.doi.org/10.1186/s12876-017-0628-3
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