AL-Kappa Primary Amyloidosis with Apolipoprotein A-IV Deposition

Intern Med. 2022;61(6):871-876. doi: 10.2169/internalmedicine.7955-21. Epub 2022 Mar 15.

Abstract

A 70-year-old woman with complaints of edema, general malaise, and hypotension was diagnosed with renal amyloidosis, and laser microdissection mass spectrometry revealed her amyloidosis to predominantly comprise the apolipoprotein A-IV type. The M-protein turned from negative to positive during the course, and a bone marrow biopsy showed smoldering myeloma. Treatment with bortezomib and dexamethasone failed to save her from heart failure six months after the onset. Western blotting of urine samples at the time of the renal biopsy showed that amyloid light-chain κ amyloidosis had been present since the onset. Unlike the myeloma, Congo red staining was positive in the plasma cells of the bone marrow.

Keywords: AL amyloidosis; Apolipoprotein A-IV amyloidosis; Congo red staining; myeloma; plasma cell.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Amyloidosis* / complications
  • Amyloidosis* / diagnosis
  • Amyloidosis* / pathology
  • Apolipoproteins A
  • Female
  • Humans
  • Immunoglobulin Light-chain Amyloidosis* / complications
  • Immunoglobulin Light-chain Amyloidosis* / diagnosis
  • Immunoglobulin Light-chain Amyloidosis* / drug therapy
  • Multiple Myeloma* / diagnosis

Substances

  • Apolipoproteins A
  • apolipoprotein A-IV