Amyloidosis natural history, complications and prognosis
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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
In amyloidosis, insoluble fibrils of amyloid are deposited in body organs, causing organ dysfunction and eventually death. Patients with amyloidosis may eventually suffer from heart failure, nephrotic syndrome, hepatomegaly and peripheral neuropathy. In primary amyloidosis the survival rate depends upon the type of organ involvement and hematological response to treatment In AL amyloidosis untreated individuals have the worst prognosis. In this group of patients median survival is one to two years.
Natural History
- In amyloidosis, insoluble fibrils of amyloid are deposited in body organs, causing organ dysfunction and eventually death.[1]
- In AL amyloidosis untreated individuals have the worst prognosis. In this group of patients median survival is one to two years.[2]
Complications
In patients with amyloidosis the most frequent complications include:[3]
Prognosis
- In primary (AL) amyloidosis survival rate depends on:[4]
- Type of organ involvement (amyloid heart disease is the main prognostic factor)
- The severity of various organ involvement
- Haematological response to treatment
- The median survival of patients with AL amyloidosis is aproximately 3.8 years.[5]
- 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
- ↑ Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
- ↑ Khan MF, Falk RH (November 2001). "Amyloidosis". Postgrad Med J. 77 (913): 686–93. PMC 1742163. PMID 11677276.
- ↑ Jerzykowska S, Cymerys M, Gil LA, Balcerzak A, Pupek-Musialik D, Komarnicki MA (2014). "Primary systemic amyloidosis as a real diagnostic challenge - case study". Cent Eur J Immunol. 39 (1): 61–6. doi:10.5114/ceji.2014.42126. PMC 4439975. PMID 26155101.
- ↑ 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.
- ↑ 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.