Supportive Care for Patients with Systemic Light Chain Amyloidosis

Hematol Oncol Clin North Am. 2020 Dec;34(6):1177-1191. doi: 10.1016/j.hoc.2020.08.007.

Abstract

Light chain amyloidosis is a disease in which clonal plasma cells produce toxic immunoglobulin light chains that form amyloid fibrils with deposition in organs, most commonly the heart and kidneys, but also the nervous system, gastrointestinal tract, and soft tissues. Treatment directed at the clonal cells eliminates light chain production and further deposition and may enable organ improvement and decrease the risk of organ failure. Supportive care manages the symptoms of organ involvement and the side effects of treatment. Supportive care also addresses the psychological and social issues that may arise in patients with light chain amyloidosis.

Keywords: Cardiac amyloidosis; Factor X deficiency; Light chain amyloidosis; Nephrotic syndrome; Peripheral neuropathy; Supportive care.

Publication types

  • Review

MeSH terms

  • Amyloid / metabolism
  • Gastrointestinal Diseases / etiology
  • Gastrointestinal Diseases / metabolism
  • Gastrointestinal Diseases / therapy*
  • Heart Diseases / etiology
  • Heart Diseases / metabolism
  • Heart Diseases / therapy*
  • Humans
  • Immunoglobulin Light Chains / metabolism
  • Immunoglobulin Light-chain Amyloidosis / complications
  • Immunoglobulin Light-chain Amyloidosis / metabolism
  • Immunoglobulin Light-chain Amyloidosis / therapy*
  • Kidney Diseases / etiology
  • Kidney Diseases / metabolism
  • Kidney Diseases / therapy*
  • Nervous System Diseases / etiology
  • Nervous System Diseases / metabolism
  • Nervous System Diseases / therapy*

Substances

  • Amyloid
  • Immunoglobulin Light Chains