Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency

Intern Med. 2019 Oct 15;58(20):3039-3043. doi: 10.2169/internalmedicine.2864-19. Epub 2019 Jun 27.

Abstract

Severe hepatic failure is rarely a cause of death in patients with immunoglobulin light chain (AL) amyloidosis. We herein report a case of AL amyloidosis involving a bleeding tendency due to factor X deficiency and marked hepatic involvement of amyloidosis. The patient died due to severe liver dysfunction two weeks after admission. The diagnosis was confirmed histologically by AL-λ amyloidosis, with the liver and spleen as the main lesions, on an autopsy. As treatment-related toxicity is strong in advanced cases, appropriate treatments are required to improve the prognosis of AL amyloidosis with severe liver dysfunction.

Keywords: AL amyloidosis; bleeding tendency; factor X; hepatic amyloidosis; liver dysfunction.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Factor X Deficiency / diagnosis
  • Factor X Deficiency / etiology*
  • Fatal Outcome
  • Female
  • Humans
  • Immunoglobulin Light Chains / metabolism*
  • Immunoglobulin Light-chain Amyloidosis / complications*
  • Immunoglobulin Light-chain Amyloidosis / diagnosis
  • Liver Failure / diagnosis
  • Liver Failure / etiology*

Substances

  • Immunoglobulin Light Chains