[Current diagnostic and therapy of light chain amyloidosis]

Pathologe. 2009 May;30(3):205-11. doi: 10.1007/s00292-009-1132-5.
[Article in German]

Abstract

Amyloidoses are protein-folding disorders in which soluble proteins are deposited as insoluble fibrillar aggregates due to a change in protein conformation. This might occur intra- or extracellularly, systemically or in a localized manner. The light chain type is the most common form of systemic amyloidoses and has the worst prognosis. The underlying disease is a monoclonal, mostly non-malignant plasma cell disorder. The causative treatment is the reduction of the amyloidogenic light chains with conventional or high-dose chemotherapy. Meanwhile, the"new drugs" used in multiple myeloma are also successfully applied. Early diagnosis is important to be able to treat patients effectively and to avoid further deterioration of organ function. Patients with newly diagnosed amyloidosis should be referred to a specialized center for consultation, diagnosis and treatment recommendation.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Amyloid / analysis*
  • Amyloidosis / drug therapy
  • Amyloidosis / pathology*
  • Antineoplastic Agents / therapeutic use
  • Bone Marrow / pathology
  • Diagnosis, Differential
  • Dose-Response Relationship, Drug
  • Early Diagnosis
  • Humans
  • Immunoglobulin Light Chains / analysis*
  • Multiple Myeloma / drug therapy
  • Multiple Myeloma / pathology
  • Paraproteinemias / drug therapy
  • Paraproteinemias / pathology*
  • Plasma Cells / pathology
  • Protein Folding / drug effects
  • Referral and Consultation

Substances

  • Amyloid
  • Antineoplastic Agents
  • Immunoglobulin Light Chains
  • amyloid protein AL