Orphanet: Primary systemic amyloidosis

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Primary systemic amyloidosis

Disease definition

Primary systemic amyloidosis (PSA) is a form of AL amyloidosis (see this term) caused by the aggregation and deposition of insoluble amyloid fibrils derived from misfolded monoclonal immunoglobulin light chains usually produced by a plasma cell tumor (see this term) and characterized by multiple organ involvement.


Classification level: Subtype of disorder
  • Synonym(s):
    • Systemic AL amyloidosis
  • Prevalence: 1-5 / 10 000
  • Inheritance: Not applicable 
  • Age of onset: -
  • ICD-10: E85.0  E85.1  E85.2  E85.3
  • OMIM: 254500
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information

Article for general public


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