Factors impeding organ recovery despite a deep haematological response in patients with systemic AL amyloidosis

Br J Haematol. 2024 Dec;205(6):2268-2272. doi: 10.1111/bjh.19766. Epub 2024 Sep 14.

Abstract

Patients with AL amyloidosis can have persistent organ dysfunction despite achieving a haematological complete response (hemCR). We aimed to identify factors for organ non-response among 143 patients who achieved hemCR for ≥6 months. Kidney, heart and liver non-response were observed in 40/117 (34%), 19/68 (28%) and 3/17 (18%) patients respectively. Predisposing factors varied by organ system. Kidney non-responders had more advanced organ dysfunction at diagnosis, whereas heart non-responders had disproportionately more lambda-typic amyloidogenic light chains. Most patients without an apparent reason for organ non-response had detectable residual clonal disease. The interplay of factors impeding organ recovery in AL amyloidosis is complex.

Keywords: AL amyloidosis; light chain; measurable residual disease; organ dysfunction; organ response.

MeSH terms

  • Adult
  • Aged
  • Female
  • Humans
  • Immunoglobulin Light-chain Amyloidosis* / blood
  • Immunoglobulin Light-chain Amyloidosis* / complications
  • Male
  • Middle Aged