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Title: Multidisciplinary supportive care in systemic light chain amyloidosis
Authors: Maroun, Bou Zerdan
Allam, Sabine
Chaulagain, Chakra P
Affiliations: Faculty of Medicine 
Keywords: Amyloidosis
Light chain
Multidisciplinary approach
Supportive care
Issue Date: 2022-06-30
Publisher: National Library of Medicine
Part of: Blood Research
Volume: 57
Issue: 2
Start page: 106
End page: 116
The immunoglobulin light-chain amyloidosis is a multisystemic disease which manifests by damage to the vital organs by light chain-derived amyloid fibril. Traditionally, the treatment has been directed to the underlying plasma cell clone with or without high dose chemotherapy followed by autologous stem cell transplantation using melphalan based conditioning. Now with the approval of highly tolerable anti-CD38 monoclonal antibody daratumumab based anti-plasma cell therapy in 2021, high rates of hematologic complete responses are possible even in patients who are otherwise deemed not a candidate for autologous stem cell transplantation. However, despite the progress, there remains a limitation in the strategies to improve symptoms particularly in patients with advanced cardiac involvement, those with nephrotic syndrome and autonomic dysfunction due to underlying systemic AL amyloidosis. The symptoms can be an ordeal for the patients and their caregivers and effective strategies are urgently needed to address them. The supportive care is aimed to counteract the symptoms of the disease and the effects of the treatment on involved organs' function and preserve patients' quality of life. Here we discuss multidisciplinary approach in a system-based fashion to address the symptom management in this dreadful disease. In addition to achieving excellent anti-plasma cell disease control, using treatment directed to remove amyloid from the vital organs can theoretically hasten recovery of the involved organs thereby improving symptoms at a faster pace. Ongoing phase III clinical trials of CAEL-101 and Birtamimab will address this question.
ISSN: 2287-979X
DOI: 10.5045/br.2022.2021227
Open URL: Link to full text
Type: Journal Article
Appears in Collections:Faculty of Medicine

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