Systemic Amyloidosis Due to Clonal Plasma Cell Diseases

Giada Bianchi, Shaji Kumar

Research output: Contribution to journalReview articlepeer-review

Abstract

Immunoglobulin light chain amyloidosis is the most common systemic amyloidosis. The pathogenetic mechanism is deposition of fibrils of misfolded immunoglobulin free light chains, more often lambda, typically produced by clonal plasma cells. Distinct Ig light chain variable region genotypes underlie most light chain amyloidosis and dictate tissue tropism. Light chain amyloidosis fibrils cause distortion of the histologic architecture and direct cytotoxicity, leading to rapidly progressive organ dysfunction and eventually patient demise. A high index of clinical suspicion with rapid tissue diagnosis and commencement of combinatorial, highly effective cytoreductive therapy is crucial to avoid irreversible organ damage and early mortality.

Original languageEnglish (US)
Pages (from-to)1009-1026
Number of pages18
JournalHematology/Oncology Clinics of North America
Volume34
Issue number6
DOIs
StatePublished - Dec 2020

Keywords

  • AL amyloidosis
  • Amyloid fibrils
  • MGUS
  • Multiple myeloma
  • Plasma cell disorders

ASJC Scopus subject areas

  • Hematology
  • Oncology

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