Cardiac amyloidosis pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor: Cafer Zorkun, M.D., Ph.D. [2]; Lakshmi Gopalakrishnan, M.B.B.S. [3]
Pathophysiology
- Amyloidosis is characterized by the deposition and extracellular accumulation of fibrillary proteins, leading to the loss of normal tissue architecture.[1]
- Most frequent types are:
- Acquired monoclonal immunoglobulin light-chain amyloidosis (AL)
- the immunoglobulin lights chains of fibrillary deposits are produced by the clonal plasma cells in the bone marrow.
- Hereditary transthyretin (TTR)-related form (ATTRm)
- caused by greater than 100 mutations of TTR
- the protein is synthesized by the liver
- Non-mutant TTR-related amyloidosis (ATTRwt)
- also know as systemic senile amyloidosis
- elderly male prepondrance
Histopathological Findings
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Amyloidosis Lesion In Left Atrium: Gross natural color view of a diagnostic lesion
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Amyloidosis Lesion In Left Atrium: Gross natural color close-up
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Heart: Perivascular amyloid, amyloidosis, congo red showing birefringence
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Heart: Perivascular amyloid, amyloidosis (Hematoxylin and eosin staining)
References
- ↑ Merlini G, Bellotti V (2003). "Molecular mechanisms of amyloidosis". The New England Journal of Medicine. 349 (6): 583–96. doi:10.1056/NEJMra023144. PMID 12904524. Retrieved 2012-02-13. Unknown parameter
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ignored (help) - ↑ Falk RH, Comenzo RL, Skinner M (1997). "The systemic amyloidoses". The New England Journal of Medicine. 337 (13): 898–909. doi:10.1056/NEJM199709253371306. PMID 9302305. Retrieved 2012-02-13. Unknown parameter
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ignored (help) - ↑ Gertz MA, Lacy MQ, Dispenzieri A (1999). "Amyloidosis: recognition, confirmation, prognosis, and therapy". Mayo Clinic Proceedings. Mayo Clinic. 74 (5): 490–4. doi:10.4065/74.5.490. PMID 10319082. Retrieved 2012-02-13. Unknown parameter
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ignored (help)