Options for Chemotherapy and Scoring Response and Relapse.

Chemotherapy for amyloid light chain (AL) amyloidosis has evolved over the years. Although high-dose melphalan and stem cell transplantation remain the standard of care for eligible patients, a vast majority of the patients at the time of presentation are not eligible for this approach and require low-intensity but highly effective induction therapy, usually based on bortezomib. Immunomodulatory agents are not well tolerated, particularly by patients with AL amyloidosis cardiomyopathy, and are reserved for second-line or later therapy. Because there currently is no Food Drug and Administration-approved therapy, participation in well-designed clinical trials of high scientific merit should be considered.