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Clinical features, laboratory characteristics, and outcomes of patients with renal vs cardiac light chain amyloidosis

British Journal of Haematology Mar 10, 2019

Sidana S, et al. - A sum of 1077 newly diagnosed AL amyloidosis cases associated with renal involvement (n=229), both cardiac and renal involvement (n=443) and cardiac involvement (n=405), were evaluated to assess the clinical characteristics, laboratory components and consequences of subjects with renal vs cardiac light chain amyloidosis. The lowest proportion of subjects with ≥ 10% bone marrow plasma cells (BMPCs) was observed in renal only patients. They noticed median overall survival (OS) across the 3 groups ie, 83 vs 19 vs 16 months in subjects not undergoing transplant whereas 5-year overall survival (OS) in patients undergoing transplant was 90% vs 75% vs 64%, respectively. They suggested a significant role of elevated sensitivity of kidney to light chains (given important synergies with renal tubular system and secretion of modified light chain products) in the pathogenesis of renal AL amyloidosis.
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