P339 LIGHT–CHAIN CARDIAC AMYLOIDOSIS WITH FULMINANT EVOLUTION, A NOT–SO–RARE CONDITION: THE IMPORTANCE OF EARLY DIAGNOSIS

Abstract Introduction Amyloid AL results from misfolding of immunoglobulin light chain (kappa or lambda) secreted by abnormal plasma cells such as in multiple myeloma. It is a rare, multisystemic disease with an estimated annual incidence rate 6 to 10 cases per million inhabitans Western countries. The prognosis this hematologic neoplasm guided the extent cardiac involvement; if untreated, survival less than months; early initiation chemotherapy treatment, median can range 4 years, except for high–risk cases. Case report: 58–year–old patient presented emergency department May 2022 first syncopal episode and sphincter release. had no cardiovascular risk factors. Echocardiografy showed markedly hypertrophic left ventricle hyper–reflective walls granular–sparkling appearance. Electromyography revealed sensory–motor polyneuropathy, early–stage autonomic neuropathy bilateral carpal tunnel syndrome. Implantation loop recorder was performed investigate cause syncope. In suspected amyloidosis, underwent electrophoresis, which monoclonal peaks, serum urine kappa lambda chains were negative. Bone scintigraphy osteotropic radiopharmaceutical uptake heart. Cardiac magnetic resonance imaging (MRI) biventricular phenocopy (concentric hypertrophy) diffuse edema–like tissue alterations. asymptomatic. August 2022, evaluation band repeated immunofixation bone marrow biopsy 9–10% cells. referred specialized center where peri–umbilical fat confirm diagnosis initiate therapy. October due recurrent syncope, advanced atrioventricular block extreme bradycardia at recorder, dual–chamber pacemaker implanted. A few days after discharge, suffered arrest home electromechanical dissociation died, only 5 months symptom. Conclusion amyloidosis aggressive hematological neoplasm, involvement. Early crucial timely therapy, means available increase survival. important maintain high level awareness diagnostic suspicion condition.