AL Amyloidosis: The Effect of Maintenance Therapy on Autologous Stem Cell Transplantation Outcomes

Abstract

Background: Autologous stem cell transplantation (ASCT) remains an effective treatment option for many patients with systemic light chain (AL) amyloidosis. While maintenance post ASCT in multiple myeloma is now standard, the decision to utilize maintenance in AL amyloidosis remains largely unexplored. The present study aims to determine the prognostic significance of utilizing maintenance therapy following ASCT and assess the impact of fluorescent in situ hybridization (FISH) abnormalities, bone marrow plasma cell burden (BMPC), and degree of organ involvement on this decision. Methods and results: This is a retrospective analysis of fifty AL amyloidosis patients who underwent ASCT at The Ohio State University. Twenty-eight patients received maintenance and twenty-two did not. Kaplan–Meier survival analysis was used to compare the effect of maintenance therapy with no significant difference in PFS (p = 0.66) and OS (p = 0.32) between the two groups. There was no difference in survival based on maintenance when further categorized by FISH, PFS (p = 0.15), and OS (p = 0.65); BMPC ≥ 10%, PFS (p = 0.49), and OS (p = 0.32); or with 2 or more organs involved, PFS (p = 0.34) and OS (p = 0.80). Conclusion: Maintenance therapy post ASCT did not impact PFS or OS when categorized by FISH abnormalities, increasing BMPC, or ≥2 organs involved in AL amyloidosis patients.

Hepatic AL Amyloidosis Without Significant Light Chain Elevation in a Patient Treated with CyBorD Plus Daratumumab

“…The optimal timing of AL amyloidosis treatment remains unclear, with some advocating delaying treatment until the patient experiences organ progression or symptomatic relapse [ 16 ]. Moreover, a recent retrospective study suggests that lenalidomide or bortezomib maintenance post-transplant did not significantly improve overall survival or progression-free survival [ 17 ]. Ultimately, whether our patient pursues maintenance therapy will be informed by clinical judgement and shared decision making.…”

An eye‐catching atypical illustration of the evaluation and management of AL amyloidosis secondary to myeloma

“…He was started on lenalidomide maintenance therapy, which, in contrast to multiple myeloma in which post‐ASCT maintenance therapy is the standard of care, has not been completely explored in the setting of AL amyloidosis. In the setting of AL amyloidosis, varying evidence exists, but some studies have shown that there is no significant difference between patients that do and do not receive maintenance therapy as measured by FISH bone marrow plasma cell burden (BMPC), progression‐free survival (PFS), and overall survival (OS) 15 …”

A Short Review of Hematopoietic Transplantation for AL Amyloidosis

“…Of this series, 28 patients received post-ABMT maintenance therapy and 22 patients did not. Kaplan-Meier analysis was employed to examine the effect of maintenance versus no maintenance, and found no statistical difference between the groups with respect to either progression-free survival or with respect to overall survival [29]. However, a number of clinical trials have been ongoing to examine the role of post-transplant maintenance therapy.…”