The Challenges in Chemotherapy and Stem Cell Transplantation for AL Amyloidosis

Publication date: Available online 9 December 2019Source: Canadian Journal of CardiologyAuthor(s): Eli Muchtar, Grace Lin, Martha GroganAbstractLight chain (AL) amyloidosis is a systemic syndrome characterized by progressive organ dysfunction leading to organ failure and death. The heart is the most commonly involved organ and the leading determinant of short and long-term survival. Pathogenic free light chains, fragments of intact immunoglobulins, are the amyloidogenic protein and are secreted by clonal bone marrow plasma cells. The goal of therapy is to cut-off the supply of these pathogenic light chains to allow organ recovery. Therapies for AL amyloidosis are based on therapies being used to treat multiple myeloma, a more common plasma cell disorder. However, as patients with AL amyloidosis have organ dysfunction, including multi-organ involvement, these patients have poor treatment tolerance and increased treatment toxicity. Unfortunately the consequences of difficulty tolerating treatment and toxicity may result in a fatal outcome. Therefore, treatment should balance the goal of achieving a rapid and meaningful reduction of the circulating light chains, while maximizing patient safety. This approach is best achieved by multidisciplinary approach involving related medical disciplines. This review describes the challenges of managing AL patients and provides a guide for physicians with a specific focus on cardiac management. We address the role of autologous stem cell tra...
Source: Canadian Journal of Cardiology - Category: Cardiology Source Type: research