1 Introduction
Systemic light chain amyloidosis (AL) occurs due to a clonal plasma cell dyscrasia, typically with multi-system involvement and associated with a poor prognosis without treatment. Cardiac involvement occurs in approximately 60%–70% of patients [1], and is the primary driver of prognosis, with advanced cardiac involvement associated with significant short-term mortality without therapy (∼ 6 months). Diagnostic delay is common, due in part to the non-specific nature of symptoms, and a high degree of clinical suspicion is required to avoid delays in diagnosis and the ensure the early commencement of therapy. In recent years, novel therapies such as the anti CD38 monoclonal antibody daratumumab has transformed the treatment landscape, providing high rates of deep and durable hematologic responses, with 78% of patients achieving at least a “very good partial response” (VGPR) with consequent improvement in organ responses and survival [2].