The Spectrum of Monoclonal Immunoglobulin-Associated Diseases

Hematol Oncol Clin North Am. 2020 Dec;34(6):997-1008. doi: 10.1016/j.hoc.2020.07.002. Epub 2020 Oct 1.

Abstract

The spectrum of immunoglobulin paraprotein-associated diseases requiring therapy extends beyond multiple myeloma and AL amyloidosis. Awareness of these is essential in ensuring timely accurate diagnosis and appropriate treatment. As most paraprotein-associated diseases are fairly uncommon, therapeutic decisions must often be made in the absence of data from randomized controlled trials. Treatment is generally directed at the underlying clonal cell population. This review focuses on the spectrum of the less common paraprotein-associated disorders. In most instances, the monoclonal immunoglobulin plays a direct role in the pathophysiology of the disease course; in a select few, the paraprotein may be a disease marker.

Keywords: Monoclonal gammopathies; Monoclonal plasma cell diseases; Nonamyloid diseases.

Publication types

  • Review

MeSH terms

  • Humans
  • Immunoglobulin Light-chain Amyloidosis* / metabolism
  • Immunoglobulin Light-chain Amyloidosis* / pathology
  • Immunoglobulin Light-chain Amyloidosis* / therapy
  • Monoclonal Gammopathy of Undetermined Significance* / metabolism
  • Monoclonal Gammopathy of Undetermined Significance* / pathology
  • Monoclonal Gammopathy of Undetermined Significance* / therapy
  • Multiple Myeloma* / metabolism
  • Multiple Myeloma* / pathology
  • Multiple Myeloma* / therapy