AL Amyloidosis

What is AL Amyloidosis?

A plasma cell disorder that causes the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains usually produced by a plasma cell tumor. It usually presents as primary systemic amyloidosis (PSA) with multiple organ involvement and less frequently as primary localized amyloidosis (PLA) restricted to a single organ.

Other condition names

  • Light-chain amyloidosis
  • Primary amyloidosis

Inheritance type

Not applicable

Prevalence

  • Worldwide: 1-9 in 100,000
  • Europe: 1-5 in 10,000

Age of Onset

  • Adult
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.