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Case report
peer-reviewed

A Plasma Cell Dyscrasia Presenting as Amyloid Cardiomyopathy and Autonomic Dysfunction in a Healthy Patient



Abstract

Systemic amyloidosis is a rare multisystem disease caused by incorrectly folded proteins that deposit pathologically in different tissues and organs of the human body. It has a very wide spectrum of clinical presentations according to the affected organ(s), and its diagnosis is commonly delayed. Cardiac involvement is the leading cause of morbidity and mortality and carries a poor prognosis, especially in primary light chain amyloidosis. Therefore any delay in the diagnosis can result in devastating outcomes for the patient. We report the case of a 65-year-old man who presented with dizziness and lightheadedness. He was found to have orthostatic hypotension and further investigations revealed the diagnosis of amyloid cardiomyopathy complicating a plasma cell dyscrasia. What is worth noting, in this case, is that the patient had cardiac amyloidosis presenting primarily as autonomic dysfunction and orthostatic hypotension, without any cardiac-specific symptoms such as heart failure or angina. This is a very unusual presentation of advanced-stage cardiac amyloidosis. This article highlights the variety of clinical presentations of cardiac amyloidosis, and focuses on the recent progress such as novel diagnostic and surveillance approaches using imaging, biomarkers, and new histological typing techniques. Current and future promising treatment options are also discussed, including methods directly targeting the amyloid deposits.



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Case report
peer-reviewed

A Plasma Cell Dyscrasia Presenting as Amyloid Cardiomyopathy and Autonomic Dysfunction in a Healthy Patient


Author Information

Rabih Tabet Corresponding Author

Internal Medicine, Staten Island University Hospital, Northwell Health

Julie Zaidan

Internal Medicine, Staten Island University Hospital, Northwell Health

Boutros Karam

Internal Medicine, Staten Island University Hospital, Northwell Health

Samer Saouma

Internal Medicine, Staten Island University Hospital

Internal Medicine, Staten Island University Hospital, Northwell Health

Foad Ghavami

Cardiology, Staten Island University Hospital, Northwell Health


Ethics Statement and Conflict of Interest Disclosures

Human subjects: Consent was obtained by all participants in this study. Conflicts of interest: The authors have declared that no conflicts of interest exist.


Case report
peer-reviewed

A Plasma Cell Dyscrasia Presenting as Amyloid Cardiomyopathy and Autonomic Dysfunction in a Healthy Patient


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Case report
peer-reviewed

A Plasma Cell Dyscrasia Presenting as Amyloid Cardiomyopathy and Autonomic Dysfunction in a Healthy Patient

  • Author Information
    Rabih Tabet Corresponding Author

    Internal Medicine, Staten Island University Hospital, Northwell Health

    Julie Zaidan

    Internal Medicine, Staten Island University Hospital, Northwell Health

    Boutros Karam

    Internal Medicine, Staten Island University Hospital, Northwell Health

    Samer Saouma

    Internal Medicine, Staten Island University Hospital

    Internal Medicine, Staten Island University Hospital, Northwell Health

    Foad Ghavami

    Cardiology, Staten Island University Hospital, Northwell Health


    Ethics Statement and Conflict of Interest Disclosures

    Human subjects: Consent was obtained by all participants in this study. Conflicts of interest: The authors have declared that no conflicts of interest exist.

    Acknowledgements


    Article Information

    Published: June 29, 2017

    DOI

    10.7759/cureus.1409

    Cite this article as:

    Tabet R, Zaidan J, Karam B, et al. (June 29, 2017) A Plasma Cell Dyscrasia Presenting as Amyloid Cardiomyopathy and Autonomic Dysfunction in a Healthy Patient. Cureus 9(6): e1409. doi:10.7759/cureus.1409

    Publication history

    Received by Cureus: May 16, 2017
    Peer review began: June 16, 2017
    Peer review concluded: June 18, 2017
    Published: June 29, 2017

    Copyright

    © Copyright 2017
    Tabet et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 3.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

    License

    This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Systemic amyloidosis is a rare multisystem disease caused by incorrectly folded proteins that deposit pathologically in different tissues and organs of the human body. It has a very wide spectrum of clinical presentations according to the affected organ(s), and its diagnosis is commonly delayed. Cardiac involvement is the leading cause of morbidity and mortality and carries a poor prognosis, especially in primary light chain amyloidosis. Therefore any delay in the diagnosis can result in devastating outcomes for the patient. We report the case of a 65-year-old man who presented with dizziness and lightheadedness. He was found to have orthostatic hypotension and further investigations revealed the diagnosis of amyloid cardiomyopathy complicating a plasma cell dyscrasia. What is worth noting, in this case, is that the patient had cardiac amyloidosis presenting primarily as autonomic dysfunction and orthostatic hypotension, without any cardiac-specific symptoms such as heart failure or angina. This is a very unusual presentation of advanced-stage cardiac amyloidosis. This article highlights the variety of clinical presentations of cardiac amyloidosis, and focuses on the recent progress such as novel diagnostic and surveillance approaches using imaging, biomarkers, and new histological typing techniques. Current and future promising treatment options are also discussed, including methods directly targeting the amyloid deposits.



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Create a free account to continue reading this article.

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Rabih Tabet

Internal Medicine, Staten Island University Hospital, Northwell Health

For correspondence:
rtabet@northwell.edu

Julie Zaidan

Internal Medicine, Staten Island University Hospital, Northwell Health

Boutros Karam

Internal Medicine, Staten Island University Hospital, Northwell Health

Samer Saouma

Internal Medicine, Staten Island University Hospital

Foad Ghavami

Cardiology, Staten Island University Hospital, Northwell Health

Rabih Tabet

Internal Medicine, Staten Island University Hospital, Northwell Health

For correspondence:
rtabet@northwell.edu

Julie Zaidan

Internal Medicine, Staten Island University Hospital, Northwell Health

Boutros Karam

Internal Medicine, Staten Island University Hospital, Northwell Health

Samer Saouma

Internal Medicine, Staten Island University Hospital

Foad Ghavami

Cardiology, Staten Island University Hospital, Northwell Health