Treatment of immunoglobulin light chain amyloidosis

Abstract

No therapy is uniformly effective in the management of immunoglobulin light chain amyloidosis (AL amyloidosis). Despite the common generalization, therapy is highly effective. Options available to patients with AL amyloidosis include high-dose therapy, but this is applicable to only about one fourth of patients. Therapies shown to be effective are based on alkylators, dexamethasone, or combinations of an alkylator and steroids. In the past 5 years, novel agents previously shown to be effective in multiple myeloma (eg, thalidomide, lenalidomide, and bortezomib) have been shown to have efficacy in the management of AL amyloidosis. Predictors of outcome include the serum brain natriuretic peptide, the number of organs involved, and the severity of cardiac involvement detected by echocardiography. Virtually all patients are candidates for a trial of therapy, and it is possible to find a nontoxic regimen that can be administered to virtually any patient.