Systemic Amyloidosis due to Low-Grade Lymphoma

Hematol Oncol Clin North Am. 2020 Dec;34(6):1027-1039. doi: 10.1016/j.hoc.2020.08.016. Epub 2020 Oct 1.


Lymphoma-related amyloidosis is a rare entity. Systemic AL amyloidosis is generally caused by an underlying plasma cell clone in the bone marrow with an intact monoclonal immunoglobulin G (IgG) or IgA protein. The rarity of the lymphoma-related amyloidosis makes the generation of data in randomized trials and the determination of the optimal treatment almost impossible. Therefore, treatment recommendations discussed here are based on either retrospective or small prospective trials of single centers.

Keywords: IgM amyloidosis; Lymphoma-related AL amyloidosis; Rituximab; Waldenstrom macroglobulinemia.

Publication types

  • Review

MeSH terms

  • Humans
  • Immunoglobulin Heavy Chains / metabolism
  • Immunoglobulin Light Chains / metabolism*
  • Immunoglobulin Light-chain Amyloidosis* / etiology
  • Immunoglobulin Light-chain Amyloidosis* / metabolism
  • Immunoglobulin Light-chain Amyloidosis* / pathology
  • Neoplasm Proteins / metabolism*
  • Plasma Cells* / metabolism
  • Plasma Cells* / pathology
  • Waldenstrom Macroglobulinemia* / complications
  • Waldenstrom Macroglobulinemia* / metabolism
  • Waldenstrom Macroglobulinemia* / pathology


  • Immunoglobulin Heavy Chains
  • Immunoglobulin Light Chains
  • Neoplasm Proteins