Incidence in USA: 1:100,000

Race with highest prevalence: Unknown

Increased risk of periop renal failure, cardiomyopathy (arrhythmias and ventricular dysfunction), bleeding from coagulopathy

Autonomic neuropathy

Increased risk of periop renal failure, cardiomyopathy (arrhythmias and ventricular dysfunction), bleeding from coagulopathy

Autonomic neuropathy

Signs of CHF

Dysrhythmias

Decreasing urine output

Extracellular deposition of amyloid-type proteins.

Congo-red stain of tissue reveals green birefringence in a polarizing microscope.

Associated end-stage renal, myocardial, and neuropathic disease.

Best diagnosed by subcutaneous abdominal fat pad aspirate or rectal biopsy.

Both acquired and hereditary forms exist.

Acquired forms are categorized as primary (AL), associated with plasma cell disorders (i.e., multiple myeloma), and secondary (AA), associated with inflammatory and infectious diseases (e.g., osteomyelitis, rheumatoid arthritis).

Hereditary forms very rare.

Acquired: Treatment of primary (AL) amyloidosis is directed at the underlying plasma cell disorder (e.g., chemotherapy, stem cell transplant). Treatment of secondary (AA) amyloidosis is directed at underlying infection/inflammation.

Hereditary: Colchicine, liver transplantation.

Treatments to clear amyloid deposits are being developed.