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Amyloid light-chain amyloidosis presenting as abdominal bloating: a case report

BACKGROUND: We present a case of amyloid light-chain amyloidosis with occult plasma cell dyscrasia, with the rare initial presentation of gastroparesis. While amyloidosis is known to affect the gastrointestinal system, rarely do patients present with gastrointestinal symptoms as their first symptom....

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Autores principales: Lee, Audry S. Y., Lee, Damian Z. Q., Vasanwala, Farhad F.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4812605/
https://www.ncbi.nlm.nih.gov/pubmed/27026530
http://dx.doi.org/10.1186/s13256-016-0857-z
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author Lee, Audry S. Y.
Lee, Damian Z. Q.
Vasanwala, Farhad F.
author_facet Lee, Audry S. Y.
Lee, Damian Z. Q.
Vasanwala, Farhad F.
author_sort Lee, Audry S. Y.
collection PubMed
description BACKGROUND: We present a case of amyloid light-chain amyloidosis with occult plasma cell dyscrasia, with the rare initial presentation of gastroparesis. While amyloidosis is known to affect the gastrointestinal system, rarely do patients present with gastrointestinal symptoms as their first symptom. To the best of our knowledge, this is the first such case reported with a definitive diagnosis made on gastroscopy. CASE PRESENTATION: A 52-year-old Malay man with abdominal bloating, early satiety, and weight loss was found to have significant gastroparesis. He had a past medical history of stable non-ischemic cardiomyopathy. Results from initial screening were negative for common causes of gastroparesis, such as diabetes or offending medications. Gastroscopy did not show any mechanical gastric outlet obstruction. Our patient subsequently developed symptoms of postural giddiness, which then prompted further investigations for possible autonomic dysfunction. These finally revealed evidence of systemic involvement, including postural hypotension, speckled myocardium with infiltrative cardiomyopathy on a transthoracic echocardiogram, and multifocal motor neuropathy on nerve conduction studies, from which he had been relatively asymptomatic. These findings were collectively suggestive of infiltrative disease. Retrospective Congo red staining of a gastric biopsy specimen confirmed the diagnosis of gastric amyloidosis. The final diagnosis was amyloid light-chain amyloidosis secondary to plasma cell dyscrasia, which was confirmed by bone marrow examination. Our patients was started on chemotherapy and prokinetic agents, with some improvement in gastrointestinal symptoms on follow-up. CONCLUSION: We present this case to highlight that, although rare, gastroparesis can be the initial sole presentation of amyloidosis. It is important for the internist, gastroenterologist, and hematologist to consider amyloidosis as a differential diagnosis in the investigation of gastroparesis and to be vigilant in monitoring for other systemic involvement.
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spelling pubmed-48126052016-03-31 Amyloid light-chain amyloidosis presenting as abdominal bloating: a case report Lee, Audry S. Y. Lee, Damian Z. Q. Vasanwala, Farhad F. J Med Case Rep Case Report BACKGROUND: We present a case of amyloid light-chain amyloidosis with occult plasma cell dyscrasia, with the rare initial presentation of gastroparesis. While amyloidosis is known to affect the gastrointestinal system, rarely do patients present with gastrointestinal symptoms as their first symptom. To the best of our knowledge, this is the first such case reported with a definitive diagnosis made on gastroscopy. CASE PRESENTATION: A 52-year-old Malay man with abdominal bloating, early satiety, and weight loss was found to have significant gastroparesis. He had a past medical history of stable non-ischemic cardiomyopathy. Results from initial screening were negative for common causes of gastroparesis, such as diabetes or offending medications. Gastroscopy did not show any mechanical gastric outlet obstruction. Our patient subsequently developed symptoms of postural giddiness, which then prompted further investigations for possible autonomic dysfunction. These finally revealed evidence of systemic involvement, including postural hypotension, speckled myocardium with infiltrative cardiomyopathy on a transthoracic echocardiogram, and multifocal motor neuropathy on nerve conduction studies, from which he had been relatively asymptomatic. These findings were collectively suggestive of infiltrative disease. Retrospective Congo red staining of a gastric biopsy specimen confirmed the diagnosis of gastric amyloidosis. The final diagnosis was amyloid light-chain amyloidosis secondary to plasma cell dyscrasia, which was confirmed by bone marrow examination. Our patients was started on chemotherapy and prokinetic agents, with some improvement in gastrointestinal symptoms on follow-up. CONCLUSION: We present this case to highlight that, although rare, gastroparesis can be the initial sole presentation of amyloidosis. It is important for the internist, gastroenterologist, and hematologist to consider amyloidosis as a differential diagnosis in the investigation of gastroparesis and to be vigilant in monitoring for other systemic involvement. BioMed Central 2016-03-30 /pmc/articles/PMC4812605/ /pubmed/27026530 http://dx.doi.org/10.1186/s13256-016-0857-z Text en © Lee et al. 2016 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
Lee, Audry S. Y.
Lee, Damian Z. Q.
Vasanwala, Farhad F.
Amyloid light-chain amyloidosis presenting as abdominal bloating: a case report
title Amyloid light-chain amyloidosis presenting as abdominal bloating: a case report
title_full Amyloid light-chain amyloidosis presenting as abdominal bloating: a case report
title_fullStr Amyloid light-chain amyloidosis presenting as abdominal bloating: a case report
title_full_unstemmed Amyloid light-chain amyloidosis presenting as abdominal bloating: a case report
title_short Amyloid light-chain amyloidosis presenting as abdominal bloating: a case report
title_sort amyloid light-chain amyloidosis presenting as abdominal bloating: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4812605/
https://www.ncbi.nlm.nih.gov/pubmed/27026530
http://dx.doi.org/10.1186/s13256-016-0857-z
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