Primary systemic amyloidosis

Primary systemic amyloidosis

Definition

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

Also known as systemic AL amyloidosis, systemic amyloidosis, systemic Immunoglobulin Light chain amyloidosis — per MONDO

Also identified as

Affected Organs

Organ Involvement Source
Lymphoid system Disease Has Primary Anatomic Site NCIT · CC BY 4.0