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Multifocal primary amyloidosis of the airways: Case report and review of the literature
Primary localized amyloidosis of the airways is an uncommon disorder characterized by amyloid deposits in the airway mucosa. In contrast to systemic amyloidosis other organs are not involved. Among the entities of airway amyloidosis, tracheobronchial amyloidosis is comparatively the most common subt...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4501523/ https://www.ncbi.nlm.nih.gov/pubmed/26236619 http://dx.doi.org/10.1016/j.rmcr.2015.05.004 |
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author | Lang, S.M. Täuscher, D. Füller, J. Müller, A.H. Schiffl, H. |
author_facet | Lang, S.M. Täuscher, D. Füller, J. Müller, A.H. Schiffl, H. |
author_sort | Lang, S.M. |
collection | PubMed |
description | Primary localized amyloidosis of the airways is an uncommon disorder characterized by amyloid deposits in the airway mucosa. In contrast to systemic amyloidosis other organs are not involved. Among the entities of airway amyloidosis, tracheobronchial amyloidosis is comparatively the most common subtype in the lower respiratory tract and laryngeal amyloidosis in the upper respiratory tract. The pathophysiology of localized airway amyloidosis is poorly understood. The clinical presentation is variable and often non-specific. No general consensus exists with regard to optimal treatment resulting in a variety of modalities used in clinical practice to manage this disorder. We report the case of a 50 year old woman with multifocal localized amyloidosis of the tracheobronchial tree and the upper airways. Tracheobronchial amyloidosis was treated with endoscopic debulking and external beam radiation, sinunasal amyloid deposits were surgically excised and are currently under surveillance. The importance of this extremely rare case lies in the multifocal presentation of an uncommon disorder requiring a multidisciplinary approach to offer optimal treatment including external beam radiation. |
format | Online Article Text |
id | pubmed-4501523 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-45015232015-08-01 Multifocal primary amyloidosis of the airways: Case report and review of the literature Lang, S.M. Täuscher, D. Füller, J. Müller, A.H. Schiffl, H. Respir Med Case Rep Case Report Primary localized amyloidosis of the airways is an uncommon disorder characterized by amyloid deposits in the airway mucosa. In contrast to systemic amyloidosis other organs are not involved. Among the entities of airway amyloidosis, tracheobronchial amyloidosis is comparatively the most common subtype in the lower respiratory tract and laryngeal amyloidosis in the upper respiratory tract. The pathophysiology of localized airway amyloidosis is poorly understood. The clinical presentation is variable and often non-specific. No general consensus exists with regard to optimal treatment resulting in a variety of modalities used in clinical practice to manage this disorder. We report the case of a 50 year old woman with multifocal localized amyloidosis of the tracheobronchial tree and the upper airways. Tracheobronchial amyloidosis was treated with endoscopic debulking and external beam radiation, sinunasal amyloid deposits were surgically excised and are currently under surveillance. The importance of this extremely rare case lies in the multifocal presentation of an uncommon disorder requiring a multidisciplinary approach to offer optimal treatment including external beam radiation. Elsevier 2015-05-27 /pmc/articles/PMC4501523/ /pubmed/26236619 http://dx.doi.org/10.1016/j.rmcr.2015.05.004 Text en © 2015 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Case Report Lang, S.M. Täuscher, D. Füller, J. Müller, A.H. Schiffl, H. Multifocal primary amyloidosis of the airways: Case report and review of the literature |
title | Multifocal primary amyloidosis of the airways: Case report and review of the literature |
title_full | Multifocal primary amyloidosis of the airways: Case report and review of the literature |
title_fullStr | Multifocal primary amyloidosis of the airways: Case report and review of the literature |
title_full_unstemmed | Multifocal primary amyloidosis of the airways: Case report and review of the literature |
title_short | Multifocal primary amyloidosis of the airways: Case report and review of the literature |
title_sort | multifocal primary amyloidosis of the airways: case report and review of the literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4501523/ https://www.ncbi.nlm.nih.gov/pubmed/26236619 http://dx.doi.org/10.1016/j.rmcr.2015.05.004 |
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