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Current Updates on the Management of AL Amyloidosis

Systemic immunoglobulin light chain (AL) amyloidosis is a rare but fatal disease. It results from clonal proliferation of plasma cells with excessive production of insoluble misfolded proteins that aggregate in the extracellular matrix, causing damage to the normal architecture and function of vario...

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Detalles Bibliográficos
Autores principales: Elsayed, Marwa, Usher, Sara, Habib, Muhammad Hamza, Ahmed, Nausheen, Ali, Jawad, Begemann, Madeline, Shabbir, Syed Ahmed, Shune, Leila, Al-Hilli, Jaffar, Cossor, Furha, Sperry, Brett W., Raza, Shahzad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elmer Press 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8425803/
https://www.ncbi.nlm.nih.gov/pubmed/34527111
http://dx.doi.org/10.14740/jh866
Descripción
Sumario:Systemic immunoglobulin light chain (AL) amyloidosis is a rare but fatal disease. It results from clonal proliferation of plasma cells with excessive production of insoluble misfolded proteins that aggregate in the extracellular matrix, causing damage to the normal architecture and function of various organs. For decades, treatment for AL amyloidosis was based mainly on therapeutic agents previously studied for its more common counterpart, multiple myeloma. As the prevalence and incidence of AL amyloidosis have increased, ongoing research has been conducted with treatments typically used in myeloma with varying success. In this review, we focus on current treatment strategies and updates to clinical guidelines and therapeutics for AL amyloidosis.