Primary 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] Syed Hassan A. Kazmi BSc, MD [3]
Overview
In primary amyloidosis, insoluble fibrils of AL amyloid are deposited in organs, causing organ dysfunction and eventually death. Patients with primary amyloidosis may eventually suffer from heart failure, nephrotic syndrome, hepatomegaly and peripheral neuropathy. In primary amyloidosis or AL amyloidosis, the survival rate depends upon the type of organ involvement and the hematological response to treatment. In AL amyloidosis, untreated individuals have the worst prognosis. In this group of patients, the median survival is one to two years.
Natural History, Complications, and Prognosis
Natural History
- In primary amyloidosis, insoluble fibrils of AL amyloid are deposited in the 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]
- Patients with amyloidosis may eventually suffer from heart failure, nephrotic syndrome, hepatomegaly and peripheral neuropathy.
Complications
In patients with primary amyloidosis, the most frequent complications include:[3]
- Heart failure
- Nephrotic syndrome
- Hepatomegaly
- Peripheral neuropathy
- Autonomic neuropathy
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
- Hematological response to treatment
- The median survival of patients with AL amyloidosis is aproximately 3.8 years.[5]
- In primary (AL) amyloidosis 27% of the patients die within 1 year of 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.