Findings presented at the 2020 American Society of Hematology Annual Meeting & Exposition suggest that ejection fraction (EF) under 55% is an independent predictor for survival in patients with light-chain (AL) amyloidosis who underwent autologous hematopoietic cell transplantation (AHCT).
Cardiac involvement dramatically worsens prognosis for patients with amyloidosis. Many patients with AL amyloidosis present with preserved EF, which occurs when the lower left ventricle is not able to fill properly with blood. The outcomes of amyloid patients with decreased EF following AHCT is understudied.
In this retrospective study, researchers reviewed data on 716 patients with AL amyloidosis who received AHCT between 1996 and 2017. All patients received an echocardiogram prior to transplant, and EF was documented. The team evaluated outcomes and baseline characteristics of patients with an EF <55% compared with patients with an EF ≥55%. Progression-free survival (PFS) and overall survival (OS) were calculated, and univariate and multivariate analysis was performed according to variables such as age, disease stage, bone marrow plasma cell percentage (BMPC), organ involvement, melphalan conditioning, AHCT year, and EF level.
Day 100 transplant related mortality (TRM) was higher in patients with EF <55% compared with those with EF ≥55% (19% vs. 6%, respectively; P=0.0006). PFS and OS were overall shorter in patients with low EF (median PFS, 38 vs. 50 months; median OS, 72.7 vs. 144 months). EF <55% vs. ≥55% was an independent predictor of both PFS and OS (hazard ratio [HR], 1.9; P=0.003). Other predictors of PFS and OS included disease staging, AHCT year before or after 2010, and melphalan conditioning. BMPC level was predictive of OS.
“Having an EF <55% is associated with a higher day 100 TRM and is an independent predictor for PFS and OS in patients with AL amyloidosis undergoing AHCT,” the researchers concluded.