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AL amyloidosis

Disease definition

A plasma cell disorder characterized by 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.

ORPHA:85443

Classification level: Disorder
  • Synonym(s):
    • Light-chain amyloidosis
    • Primary amyloidosis
  • Prevalence: 1-5 / 10 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: E85.9
  • OMIM: 254500
  • UMLS: C0268381
  • MeSH: C531616
  • GARD: 5797
  • MedDRA: 10036673

Detailed information

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