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Light Chain Amyloidosis Presenting as a Septic Shock: A Case Report and Review of Literature

Light chain (AL) amyloidosis is a plasma cell dyscrasia that results in an overproduction of immunoglobulins of the lambda or kappa light chains. These monoclonal ALs begin to form fibrils with each other and exert their toxic effect by depositing in different organs around the body. Disease present...

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Detalles Bibliográficos
Autores principales: Bazzi, Talal, Kropman, Kory, Benjamin, Mark, Al-Rammahi, Ali
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9653032/
https://www.ncbi.nlm.nih.gov/pubmed/36381809
http://dx.doi.org/10.7759/cureus.30263
Descripción
Sumario:Light chain (AL) amyloidosis is a plasma cell dyscrasia that results in an overproduction of immunoglobulins of the lambda or kappa light chains. These monoclonal ALs begin to form fibrils with each other and exert their toxic effect by depositing in different organs around the body. Disease presentation is indistinct, but it is ideal to diagnose this disorder before end-organ damage is caused. Once the diagnosis of AL amyloidosis is confirmed, the best treatment is autologous stem cell transplantation once a candidate is deemed fit for it; however, there are other chemotherapy agents whose patients can be administered until they undergo stem cell transplantation. In this case presentation and systematic review of AL amyloidosis, we discuss a patient who presented with septic shock and further workup leading to a diagnosis of advanced-stage amyloidosis. We also take a deeper look at AL amyloidosis providing a comprehensive review of the disease process and its treatment options.