Abstract
The recent decades have ushered in considerable advancements in the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL amyloidosis-unrelated factors may impact mortality. In this study, we evaluated survival trends and primary causes of death among 2337 individuals with AL amyloidosis referred to the Boston University Amyloidosis Center. Outcomes were analyzed according to date of diagnosis: 1980-1989 (era 1), 1990-1999 (era 2), 2000-2009 (era 3), and 2010-2019 (era 4). Overall survival increased steadily with median values of 1.4, 2.6, 3.3, and 4.6 years for eras 1–4, respectively (P < 0.001). Six-month mortality decreased over time from 23% to 13%. Wide gaps in survival persisted amid patient subgroups; those with age at diagnosis ≥70 years had marginal improvements over time. Most deaths were attributable to disease-related factors, with cardiac failure (32%) and sudden unexpected death (23%) being the leading causes. AL amyloidosis-unrelated mortality increased across eras (from 3% to 16% of deaths) and with longer-term survival (29% of deaths occurring >10 years after diagnosis). Under changing standards of care, survival improved and early mortality declined over the last 40 years. These findings support a more optimistic outlook for patients with AL amyloidosis.
Highlights
Systemic light chain (AL) amyloidosis was historically regarded as an inevitably fatal disease
Options remained limited for 65–75% of newly diagnosed patients who were ineligible for high-dose melphalan and autologous stem cell transplantation (HDM/SCT)
Data source and study population Patients with AL amyloidosis diagnosed between January 1980 and December 2019 were identified from the prospectively maintained database at the Amyloidosis Center at Boston University School of Medicine and Boston Medical Center. Those with localized AL amyloidosis, myeloma-associated AL amyloidosis, and B cell lymphoproliferative disorder-associated AL amyloidosis were excluded from analysis due to
Summary
Systemic light chain (AL) amyloidosis was historically regarded as an inevitably fatal disease. A turning point came in the 1990s with the introduction of high-dose melphalan and autologous stem cell transplantation (HDM/SCT) for AL amyloidosis [4, 5]. This treatment modality offered a more favorable prognosis for carefully selected patients [6]. Beyond treatments, standardized riskstratification and treatment response assessment, owing to the advent of the serum-free light-chain assay and organ dysfunction biomarkers in the 2000s, contributed to a much-improved outlook for this rare disease [14,15,16,17,18,19]
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