Highlights & Basics
- Amyloidosis is caused by the deposition of amyloid proteins in tissue and organs. It may have a primary cause, may be inherited, or may be secondary to other diseases.
- Immunoglobulin light chain (AL) amyloidosis (also called primary systemic amyloidosis) is the most common type of amyloidosis.
- Amyloidosis usually presents with unexplained weight loss, fatigue, and edema resistant to diuretic therapy.
- Immunofixation of the serum and urine confirms the presence of monoclonal light chains in AL amyloidosis. Biopsy verification of amyloid deposits is essential. Accurate classification of amyloid deposit in tissues is necessary prior to initiating appropriate therapy.
- Treatment includes management of resulting clinical syndromes, such as nephrotic syndrome, neuropathy, cardiomyopathy, and conduction disorders.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Bilateral periorbital ecchymosis (amyloid purpura) in a patient with AL amyloidosis
Classic periorbital purpura
Macroglossia in a patient with AL amyloidosis
Congo red stain blood vessel in a bone marrow biopsy demonstrating green birefringence pathognomonic of amyloidosis
Electron micrograph demonstrating classical amyloid fibrils
Bilateral periorbital ecchymosis (amyloid purpura) in a patient with AL amyloidosis
Macroglossia in a patient with AL amyloidosis
Renal biopsy demonstrating amyloid deposits as amorphous replacement of the glomerular architecture
Citations
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Gillmore JD, Wechalekar A, Bird J, et al. Guidelines on the diagnosis and investigation of AL amyloidosis. Br J Haematol. 2015 Jan;168(2):207-18.[Abstract][Full Text]
Dispenzieri A, Buadi F, Kumar SK, et al. Treatment of immunoglobulin light chain amyloidosis: Mayo stratification of myeloma and risk-adapted therapy (mSMART) consensus statement. Mayo Clin Proc. 2015 Aug;90(8):1054-81.[Abstract][Full Text]
Adams D, Suhr OB, Hund E, et al. First European consensus for diagnosis, management, and treatment of transthyretin familial amyloid polyneuropathy. Curr Opin Neurol. 2016 Feb;29(suppl 1):S14-26.[Abstract][Full Text]
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