Monoclonal gammopathy evolving into sytemic amyloidosis with cardiac involvement. A case report

We report a case of systemic light chain amyloidosis with cardiac involvement, preceded by a monoclonal IgG lambda gammopathy. The clinical diagnosis was based on signs of heart failure, elevated biomarkers, and imaging findings. The diagnosis was confirmed by elevated light chains in the blood and...

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Detalles Bibliográficos
Autores: Valenzuela-Rodríguez, Germán, Araoz Tarco, Ofelia, Fernández Vertiz, Iván
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2025
País:Perú
Institución:Instituto Nacional Cardiovascular
Repositorio:Archivos peruanos de cardiología y cirugía cardiovascular
Idioma:español
inglés
OAI Identifier:oai:apcyccv.org.pe:article/477
Acceso en línea:https://apcyccv.org.pe/index.php/apccc/article/view/477
Access Level:acceso abierto
Palabra clave:Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas
Corazón
Gammapatía Monoclonal de Relevancia Indeterminada
Light Chain Amyloidosis
Cardiac
Monoclonal Gammopathy of Undetermined Significance
Descripción
Sumario:We report a case of systemic light chain amyloidosis with cardiac involvement, preceded by a monoclonal IgG lambda gammopathy. The clinical diagnosis was based on signs of heart failure, elevated biomarkers, and imaging findings. The diagnosis was confirmed by elevated light chains in the blood and urine, and the presence of amyloid deposits in the periumbilical fat biopsy and in different segments of the digestive tract. Treatment with daratumumab plus bortezomib plus dexamethasone was initiated, and autologous hematopoietic stem cell therapy was administered 22 months after diagnosis, with a favorable clinical outcome.