Prevalence of plasma cell and lymphoproliferative disorders among blood relatives of patients with light chain amyloidosis

Br J Haematol. 2022 Sep;198(5):861-865. doi: 10.1111/bjh.18225. Epub 2022 May 2.

Abstract

With limited existing data on hereditary factors in light chain (AL) amyloidosis, we conducted a study of patients with plasma cell dyscrasias or lymphoproliferative disorders in their family history. Among 1621 patients, we identified 44 probands (2·7%) with 52 relatives affected. The most common disorders in family members were multiple myeloma (48%) and AL amyloidosis (18%). Light chain isotype was 100% congruent in families with known clonal immunoglobulin for both members. Despite matching light chain isotype, organ involvement varied between members in families with multiple cases of AL amyloidosis. These findings help generate hypotheses about familial influences in AL amyloidosis.

Keywords: AL amyloidosis; familial risk; genetics; hereditary factors; light chain.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyloidosis* / epidemiology
  • Amyloidosis* / genetics
  • Family
  • Humans
  • Immunoglobulin Light Chains / genetics
  • Immunoglobulin Light-chain Amyloidosis* / epidemiology
  • Immunoglobulin Light-chain Amyloidosis* / genetics
  • Plasma Cells
  • Prevalence

Substances

  • Immunoglobulin Light Chains