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Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease

Amyloidosis is an uncommon disease characterized by deposition of proteinaceous material in the extracellular matrix, which results from abnormal protein folding. Even though more than 25 precursor proteins are identified, majority of systemic amyloidosis results from deposition of abnormal immunogl...

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Autores principales: Priyamvada, P. S., Morkhandikar, S., Srinivas, B. H., Parameswaran, S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4379617/
https://www.ncbi.nlm.nih.gov/pubmed/25838650
http://dx.doi.org/10.4103/0971-4065.135911
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author Priyamvada, P. S.
Morkhandikar, S.
Srinivas, B. H.
Parameswaran, S.
author_facet Priyamvada, P. S.
Morkhandikar, S.
Srinivas, B. H.
Parameswaran, S.
author_sort Priyamvada, P. S.
collection PubMed
description Amyloidosis is an uncommon disease characterized by deposition of proteinaceous material in the extracellular matrix, which results from abnormal protein folding. Even though more than 25 precursor proteins are identified, majority of systemic amyloidosis results from deposition of abnormal immunoglobulin (Ig) light chains. In heavy chain amyloidosis (AH), deposits are derived from both heavy chain alone, whereas in heavy and light chain amyloidosis (AHL), the deposits are derived from Ig heavy chains and light chains. Both AH and AHL are extremely rare diseases. Here, we report an unusual presentation of IgG (lambda) AHL amyloidosis in the background of multiple myeloma, where the initial clinical presentation was complete heart block, which preceded the definitive diagnosis by 18 months.
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spelling pubmed-43796172015-04-02 Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease Priyamvada, P. S. Morkhandikar, S. Srinivas, B. H. Parameswaran, S. Indian J Nephrol Case Report Amyloidosis is an uncommon disease characterized by deposition of proteinaceous material in the extracellular matrix, which results from abnormal protein folding. Even though more than 25 precursor proteins are identified, majority of systemic amyloidosis results from deposition of abnormal immunoglobulin (Ig) light chains. In heavy chain amyloidosis (AH), deposits are derived from both heavy chain alone, whereas in heavy and light chain amyloidosis (AHL), the deposits are derived from Ig heavy chains and light chains. Both AH and AHL are extremely rare diseases. Here, we report an unusual presentation of IgG (lambda) AHL amyloidosis in the background of multiple myeloma, where the initial clinical presentation was complete heart block, which preceded the definitive diagnosis by 18 months. Medknow Publications & Media Pvt Ltd 2015 /pmc/articles/PMC4379617/ /pubmed/25838650 http://dx.doi.org/10.4103/0971-4065.135911 Text en Copyright: © Indian Journal of Nephrology http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Priyamvada, P. S.
Morkhandikar, S.
Srinivas, B. H.
Parameswaran, S.
Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease
title Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease
title_full Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease
title_fullStr Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease
title_full_unstemmed Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease
title_short Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease
title_sort heavy and light chain amyloidosois presenting as complete heart block: a rare presentation of a rare disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4379617/
https://www.ncbi.nlm.nih.gov/pubmed/25838650
http://dx.doi.org/10.4103/0971-4065.135911
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