Amyloidosis, particularly AL (immunoglobulin light chain) amyloidosis, is a rare illness that can lead to the gradual dysfunction of organs, organ failure, and ultimately death.
When clonal plasma cells in the bone marrow release free light chains into the bloodstream, issues arise in AL amyloidosis. These light chains, which are normally part of immunoglobulins or antibodies, become misshapen and clump together to form amyloid fibrils that build up in organs and tissues.
In an article titled “Systemic Light Chain Amyloidosis,” Dr. Vaishali Sanchorawala, who heads the Amyloidosis Center at the Chobanian & Avedisian School of Medicine and Boston Medical Center, discusses recent advancements in grasping the underlying causes, symptoms, risk assessment, and treatment options of AL amyloidosis, along with future directions for both research and treatment.
Dr. Sanchorawala mentioned in her review, “There have been remarkable strides in the care of patients dealing with systemic immunoglobulin light chain (AL) amyloidosis over the last forty years, resulting in significant improvements in patient outcomes. Survival rates have noticeably increased, yet there are still plenty of unaddressed challenges.”
She pointed out that the severity of cardiac complications plays a crucial role in determining survival rates. AL amyloidosis affects the heart in 70 to 80% of patients, with cardiac issues being the primary cause of death. However, other vital organs like the kidneys, liver, and peripheral and autonomic nervous systems can also be impacted by this condition.
Treatment options involve targeting clonal plasma cells to halt light chain production and ongoing research into antifibril monoclonal antibodies that facilitate the elimination of amyloid deposits from organs.
Dr. Sanchorawala highlighted, “There is a growing array of treatments available for AL amyloidosis, providing hope for a favorable outlook and overcoming challenges for patients in 2024.” Timely detection is crucial, and she recommended evaluating patients with a range of symptoms and clinical conditions for potential AL amyloidosis.
Despite enhancements in diagnosing and treating AL amyloidosis, ongoing efforts in fundamental and clinical research are crucial to improving the future for individuals affected by this condition.
