Encyclopedia of Heart Diseases

2011 Edition
| Editors: M. Gabriel Khan


Reference work entry
DOI: https://doi.org/10.1007/978-1-60761-219-3_6


Amyloidosis is a clinical disorder caused by extracellular deposition of insoluble abnormal fibrils, derived from the aggregation of misfolded normally soluble protein (Merlini and Westermark 2004; Selkoe 2003). A systematic review of peer-reviewed publications that were identified through searches of Medline and the Cochrane Database from 1980 to June 2007 summarizes the effective clinical evaluation and management of cardiac amyloidosis (Selvanayagam et al. 2007).
  • Primary amyloidosis is now termed systemic AL amyloidosis, and cardiac involvement is not uncommon. On the other hand, secondary amyloidosis rarely affects the heart. The AL fibrils are derived from monoclonal immunoglobulin light chains. B-cell  dyscrasias, mainly benign monoclonal  gammopathies, followed by myeloma, macroglobulinemia, and lymphomas are the main causes of AL amyloidosis. Senile amyloidosis occurs mainly in individuals older than 80 years of age.

Regardless of the underlying pathogenesis of amyloid...
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Suggested Reading

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