Amiloidosi cardiaca da catene leggere: gestione multidisciplinare e innovazioni terapeutiche. Documento di consenso SIC/ANMCO della Rete Italiana dell’Amiloidosi Cardiaca (RIAC)

Light chain (AL) amyloidosis is a rare systemic disease caused by monoclonal immunoglobulin light chains with abnormal folding that aggregate into fibrils, which deposit in extracellular tissues. This process leads to cytotoxicity and organ dysfunction. Cardiac involvement is the main prognostic determinant and requires a multidisciplinary management approach. In recent years, the treatment of AL amyloidosis has significantly evolved with the introduction of innovative agents such as proteasome inhibitors, immunomodulators, and monoclonal antibodies like daratumumab, which has shown a favorable impact on hematological outcomes and organ function. The daratumumab-CyBorD regimen is currently the standard first-line therapeutic option. The therapy is tailored based on the stage of cardiac and renal damage, aiming for a complete hematological and organ response. The management of cardiac involvement, including aortic stenosis, atrial fibrillation, thromboembolic risk, conduction disorders, arrhythmias, and heart failure, plays a crucial role in prognosis. An integrated multidisciplinary approach in specialized centers experienced in the disease is essential to optimize clinical outcomes.