Multiorgan involvement by amyloid light chain amyloidosis

J Int Med Res. 2019 Apr;47(4):1778-1786. doi: 10.1177/0300060518814337. Epub 2019 Feb 25.

Abstract

Amyloid light chain (AL) amyloidosis is a protein conformational disease. AL amyloidosis results from aggregation of misfolded proteins that are deposited in tissues as amyloid fibrils. Diagnosis of AL amyloidosis can be challenging due to its low incidence and clinical complexity. Therapy requires a risk-adapted approach involving dose reductions and schedule modifications of chemotherapy regimens along with close monitoring of hematologic and organ responses. We herein describe a patient whose condition was diagnosed as systemic AL amyloidosis and presented with splenic rupture as the initial symptom. Congo red staining of the kidney biopsy was positive. The normal structure of the liver and spleen had been replaced by amyloid deposition. The chemotherapy strategy involved a combination of bortezomib, cyclophosphamide, thalidomide, and dexamethasone.

Keywords: Amyloid light chain (AL) amyloidosis; Congo red; amyloid deposition; chemotherapy; multiorgan involvement; splenic rupture.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Humans
  • Immunoglobulin Light-chain Amyloidosis / complications
  • Immunoglobulin Light-chain Amyloidosis / drug therapy
  • Immunoglobulin Light-chain Amyloidosis / pathology*
  • Liver Diseases / complications
  • Liver Diseases / drug therapy
  • Liver Diseases / pathology*
  • Male
  • Middle Aged
  • Prognosis
  • Splenic Rupture / complications
  • Splenic Rupture / drug therapy
  • Splenic Rupture / pathology*