Cardiac amyloidosis pathophysiology: Difference between revisions
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{{Cardiac amyloidosis}} | {{Cardiac amyloidosis}} | ||
{{CMG}}; '''Associate Editor:''' {{CZ}} | {{CMG}}; '''Associate Editor:''' {{CZ}}; {{LG}} | ||
==Pathophysiology== | |||
*Amyloidosis is characterized by the deposition and extracellular accumulation of fibrillary proteins, leading to the loss of normal tissue architecture.<ref name="pmid12904524">{{cite journal |author=Merlini G, Bellotti V |title=Molecular mechanisms of amyloidosis |journal=[[The New England Journal of Medicine]] |volume=349 |issue=6 |pages=583–96 |year=2003 |month=August |pmid=12904524 |doi=10.1056/NEJMra023144 |url=http://www.nejm.org/doi/abs/10.1056/NEJMra023144?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed |accessdate=2012-02-13}}</ref> | |||
*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 | |||
*All three forms frequently involve the myocardium.<ref name="pmid9302305">{{cite journal |author=Falk RH, Comenzo RL, Skinner M |title=The systemic amyloidoses |journal=[[The New England Journal of Medicine]] |volume=337 |issue=13 |pages=898–909 |year=1997 |month=September |pmid=9302305 |doi=10.1056/NEJM199709253371306 |url=http://www.nejm.org/doi/abs/10.1056/NEJM199709253371306?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed |accessdate=2012-02-13}}</ref><ref name="pmid10319082">{{cite journal |author=Gertz MA, Lacy MQ, Dispenzieri A |title=Amyloidosis: recognition, confirmation, prognosis, and therapy |journal=[[Mayo Clinic Proceedings. Mayo Clinic]] |volume=74 |issue=5 |pages=490–4 |year=1999 |month=May |pmid=10319082 |doi=10.4065/74.5.490 |url=http://linkinghub.elsevier.com/retrieve/pii/S0025-6196(11)65130-6 |accessdate=2012-02-13}}</ref> | |||
==Histopathological Findings== | ==Histopathological Findings== |
Revision as of 15:03, 13 February 2012
Cardiac amyloidosis Microchapters |
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Cardiac amyloidosis pathophysiology On the Web |
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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
-
Amyloidosis Lesion In Left Atrium: Gross natural color view of a diagnostic lesion
-
Amyloidosis Lesion In Left Atrium: Gross natural color close-up
-
Heart: Perivascular amyloid, amyloidosis, congo red showing birefringence
-
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
|month=
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
|month=
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
|month=
ignored (help)