Factors impeding organ recovery despite a deep haematological response in patients with systemic <scp>AL</scp> amyloidosis
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.
Authors
- Natasha Burke (ORCID: https://orcid.org/0009-0006-5199-1280)
- Vaishali Sanchorawala (ORCID: https://orcid.org/0000-0002-6307-2445)
- Lisa Mendelson (ORCID: https://orcid.org/0000-0003-2821-6009)
- Tracy Joshi (ORCID: https://orcid.org/0000-0003-4048-6271)
- Andrew Staron (ORCID: https://orcid.org/0000-0003-1638-4873)
Institutions
- Boston University (US)
- Boston Medical Center (US)
- Amyloidosis Foundation (US)
Publication Details
- Journal
- British Journal of Haematology
- Published
- 2024-09-14
- DOI
- https://doi.org/10.1111/bjh.19766
- Citations
- 5
- Primary Topic
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Type
- article
- Field-Weighted Citation Impact
- 1.17