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Title: Systemic AL amyloidosis: current approach and future direction
Authors: Bou Zerdan, Maroun
Nasr, Lewis
Khalid, Farhan
Allam, Sabine
Bouferraa, Youssef
Batool, Saba
Tayyeb, Muhammad
Adroja, Shubham
Mammadii, Mahinbanu
Anwer, Faiz
Raza, Shahzad
Chaulagain, Chakra P
Affiliations: Faculty of Medicine 
Keywords: Amyloidosis
Issue Date: 2023-04-26
Publisher: National Library of Medicine
Part of: Oncotarget
Volume: 14
Start page: 384
End page: 394
Systemic Light chain (AL) amyloidosis is a monoclonal plasma cell proliferative disorder characterized by deposition of amyloidogenic monoclonal light chain fragments causing organ dysfunction. It is a fatal disease and if not diagnosed and treated early can lead to organ failure and potentially death. The renal system along with the cardiovascular system are the most common organs involved but other organs such as gut and liver can be involved as well. The initial evaluation of patients requires confirming the diagnosis with tissue biopsy and staining with Congo red followed by confirmatory typing with mass spectrometry of the Congo red positive tissue. Then establishing the extent of the organs involvement by various staging and biomarkers testing. The treatment options and the tolerability of therapy depend on the disease staging, frailty, and co-morbidities. The autologous hematopoietic cell transplantation (HCT) after high dose melphalan therapy is an effective strategy which is usually done after initial bortezomib induction therapy. Unfortunately, most systemic AL amyloidosis patients are not candidate for HCT due to frailty, old age, multi-organ involvement, renal and heart failure at the time of diagnosis. While it is widely accepted that the patients need to be treated until they achieve complete hematologic response, the maintenance therapy after HCT is not well established in AL amyloidosis. In this review, we report the literature on the latest treatment updates of AL amyloidosis and the ongoing clinical trials highlighting the future treatments.
DOI: 10.18632/oncotarget.28415
Open URL: Link to full text
Type: Journal Article
Appears in Collections:Faculty of Medicine

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