Publication: Primary Systemic Amyloidosis: A Case Report
Date
2023
Article Type
Case Report
Journal Title
Journal ISSN
JNMA Print ISSN: 0028-2715; Online ISSN: 1815-672X
Volume Title
Pages
Pages: 822 - 824
Publisher
Nepal Medical Association
Abstract
Abstract
Primary systemic amyloidosis is a systemic disease characterised by the deposition of misfolded proteins extracellularly in different organs without any known cause in the background, eventually leading to multiorgan dysfunction and death. The incidence of primary amyloidosis is estimated at 5.1-12.8 cases per million, with a poor prognosis. We report a case of a 69-year male with lower back pain, shortness of breath, and anasarca diagnosed as primary systemic amyloidosis by serum-free light chain assay and kidney needle biopsy. He was started on intravenous bortezomib and dexamethasone. Though he adhered to his medications, with time he developed renal insufficiency marked by azotemia following which hemodialysis was performed. Primary systemic amyloidosis is a rare clinical condition with a very poor prognosis. Further studies are needed to understand the proper pathophysiology and treatment of the disease.
Description
Saurav Sen Oli
Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal
Abhishek Jha
Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal
Anisha Karki
Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal
Shova Sapkota
Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal
Laxman Adhikari
Department of Nephrology, Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal
Keywords
Identifier
https://doi.org/10.31729/jnma.8297