Sudden death in lambda light chain AL cardiac amyloidosis: a review of literature and update for clinicians and pathologists.

Sudden death in lambda light chain AL cardiac amyloidosis: a review of literature and update for clinicians and pathologists. Int J Clin Exp Pathol. 2020;13(7):1474-1482 Authors: D'Errico S, Mazzanti A, Baldari B, Maiese A, Frati P, Fineschi V Abstract Light chain (AL) amyloidosis is the most common type of systemic amyloidosis, affecting around 10 people per million per year. In Europe, approximately 5000 new diagnosis per year are reported. Deposition of amyloid fibrils derived from antibody light chains are key pathogenic agents in AL amyloidosis. They can be deposited in multiple organs but cardiac involvement carries a major risk of mortality. The prognosis is poor in cases associated with multiple myeloma. The average survival is around 1 year. Up to half of all patients with cardiac amyloidosis die suddenly; 75% ofthose deaths are due to heart failure. Ventricular arrhythmia is also associated with cardiac amyloidosis and unexpected death. It is crucial to make a diagnosis and start treatment at an early stage. Recent data suggest that cardiac amyloidosis has become a treatable and curable condition with a combination of agents targeting multiple steps of the amyloid cascade. ICD implantation may not be as effective for the therapy of light chain (AL) cardiac amyloidosis as supposed earlier. In cases of unexpected and sudden death, autopsy may show unknown conditions and is valuable to assess existing risks for family members....
Source: International Journal of Clinical and Experimental Pathology - Category: Pathology Authors: Tags: Int J Clin Exp Pathol Source Type: research