Encyclopedia of Heart Diseases

2011 Edition
| Editors: M. Gabriel Khan

Amyloid

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

Overview

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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Bibliography

References

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Suggested Reading

  1. Comenzo RL (2009) How I treat amyloidosis. Blood 114(15):3147–3157PubMedGoogle Scholar
  2. Rapezzi C, Guidalotti P, Salvi F et al (2008) Usefulness of 99mTc-DPD scintigraphy in cardiac amyloidosis. J Am Coll Cardiol 51:1509–1510PubMedGoogle Scholar
  3. Shah KB, Inoue Y, Mehra MR (2006) Amyloidosis and the heart: a comprehensive review. Arch Intern Med 166:1805–1813PubMedGoogle Scholar
  4. Syed IS, Glockner JF, Feng D et al (2010) Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis JACC cardiovasc imaging 3:155–164Google Scholar

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