Amyloidosis natural history, complications and prognosis: Difference between revisions

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==Prognosis==
==Prognosis==
* In primary (AL) amyloidosis survival rate depends on:<ref name="pmid22909024">{{cite journal |vauthors=Desport E, Bridoux F, Sirac C, Delbes S, Bender S, Fernandez B, Quellard N, Lacombe C, Goujon JM, Lavergne D, Abraham J, Touchard G, Fermand JP, Jaccard A |title=Al amyloidosis |journal=Orphanet J Rare Dis |volume=7 |issue= |pages=54 |date=August 2012 |pmid=22909024 |pmc=3495844 |doi=10.1186/1750-1172-7-54 |url=}}</ref>
** The spectrum of organ involvement (amyloid heart disease is the main prognostic factor)
** The severity of organs involvement
** Haematological response to treatment
* The median survival of patients with AL amyloidosis is aproximately 3.8 years.<ref name="pmid21483018">{{cite journal |vauthors=Merlini G, Seldin DC, Gertz MA |title=Amyloidosis: pathogenesis and new therapeutic options |journal=J. Clin. Oncol. |volume=29 |issue=14 |pages=1924–33 |date=May 2011 |pmid=21483018 |pmc=3138545 |doi=10.1200/JCO.2010.32.2271 |url=}}</ref>
* The median survival of patients with AL amyloidosis is aproximately 3.8 years.<ref name="pmid21483018">{{cite journal |vauthors=Merlini G, Seldin DC, Gertz MA |title=Amyloidosis: pathogenesis and new therapeutic options |journal=J. Clin. Oncol. |volume=29 |issue=14 |pages=1924–33 |date=May 2011 |pmid=21483018 |pmc=3138545 |doi=10.1200/JCO.2010.32.2271 |url=}}</ref>
* In AL amyloidosis 27% of patients dying within 1 year from diagnosis.
* In primary (AL) amyloidosis 27% of patients dying within 1 year from diagnosis.
* The major determinant of outcome in amyloidosis is the extent of cardiac involvement.
* The major determinant of outcome in amyloidosis is the extent of cardiac involvement.
** Cardiac amyloidosis is the cause of death in 75% of the patients who died, including sudden death in 25%.
** Cardiac amyloidosis is the cause of death in 75% of the patients who died, including sudden death in 25%.

Revision as of 20:28, 8 May 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]

Overview

Natural History

  • In amyloidosis, insoluble fibrils of amyloid are deposited in body organs, causing organ dysfunction and eventually death.[1]

Complications

Prognosis

  • In primary (AL) amyloidosis survival rate depends on:[2]
    • The spectrum of organ involvement (amyloid heart disease is the main prognostic factor)
    • The severity of organs involvement
    • Haematological response to treatment
  • The median survival of patients with AL amyloidosis is aproximately 3.8 years.[3]
  • In primary (AL) amyloidosis 27% of patients dying within 1 year from diagnosis.
  • The major determinant of outcome in amyloidosis is the extent of cardiac involvement.
    • Cardiac amyloidosis is the cause of death in 75% of the patients who died, including sudden death in 25%.

References

  1. Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
  2. Desport E, Bridoux F, Sirac C, Delbes S, Bender S, Fernandez B, Quellard N, Lacombe C, Goujon JM, Lavergne D, Abraham J, Touchard G, Fermand JP, Jaccard A (August 2012). "Al amyloidosis". Orphanet J Rare Dis. 7: 54. doi:10.1186/1750-1172-7-54. PMC 3495844. PMID 22909024.
  3. Merlini G, Seldin DC, Gertz MA (May 2011). "Amyloidosis: pathogenesis and new therapeutic options". J. Clin. Oncol. 29 (14): 1924–33. doi:10.1200/JCO.2010.32.2271. PMC 3138545. PMID 21483018.


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