Systemic AL amyloidosis: current approach and future direction
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Maroun Bou Zerdan1, Lewis Nasr2, Farhan Khalid3, Sabine Allam4, Youssef Bouferraa5, Saba Batool6, Muhammad Tayyeb7, Shubham Adroja8, Mahinbanu Mammadii1, Faiz Anwer9, Shahzad Raza9 and Chakra P. Chaulagain10
1 Department of Internal Medicine, SUNY Upstate Medical University, Syracuse, NY 13210, USA
2 University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
3 Department of Internal Medicine, Monmouth Medical Center, Long Branch, NJ 07740, USA
4 Department of Medicine and Medical Sciences, University of Balamand, Balamand, Lebanon
5 Department of Internal Medicine, Cleveland Clinic, Cleveland, OH 44195, USA
6 Department of Internal Medicine, UnityPoint Methodist, Peoria, IL 61636, USA
7 Department of Internal Medicine, Monmouth Medical Center, Long Branch, NJ 07740, USA
8 Hematology and Medical Oncology, Houston Methodist Cancer Center, Houston, TX 77094, USA
9 Department of Hematology-Oncology, Cleveland Clinic, Cleveland, OH 44195, USA
10 Department of Hematology-Oncology, Myeloma and Amyloidosis Program, Maroone Cancer Center, Cleveland Clinic Florida, Weston, FL 33331, USA
|Chakra P. Chaulagain,||email:||[email protected]|
Keywords: amyloidosis; management
Received: March 16, 2023 Accepted: March 28, 2023 Published: April 26, 2023
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.
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