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Hsct in Patients With Cardiac Amyloidosis Publisher



Hajahmadi M1 ; Rad S2
Authors
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Authors Affiliations
  1. 1. Cardiovascular Department, Rasoul Akram General Hospital Iran University of Medical Sciences, Tehran, Iran
  2. 2. Internal Medicine Department, Hematology and Oncology Ward, Tehran University of Medical Science Shariati General Hospital, Tehran, Iran

Source: Cardiovascular Considerations in Hematopoietic Stem Cell Transplantation Published:2024


Abstract

Cardiac Amyloidosis characterize as a syndrome due to deposition of amyloid in the extra cardiomyocyte space. It divides in two most common subtypes: AL and ATTR that are the reason of more than 95% of cases. Because of the heterogeneity in clinical presentation delay in diagnosis is common. In recent years with effective therapies, the outcomes improved and the importance of early detection and prevention of profound organ failure is further enhanced. Treatment of AL amyloidosis AL amyloidosis is complicated and The backbone of treatment is mailnly multi anti-plasma cell therapies. A growing number of studies on this syndrome are ongoing, to make treatment strategies more and more evidence-based. One of the main part of AL Amyloidosis management is supportive care. When inherent organ dysfunction (specially heart failure Heart failure), occurs effective therapy is going to have limited efficiency. High dose of Melphalan is indicated for eligible patients for stem cell transplantation, followed by stem cell rescue. ASCT is done for selected AL amyloidosis AL amyloidosis patients when therapy-related mortality is acceptable. This review brings an update summery on the diagnostic approach and treatment of cardiac amyloidosis Cardiac Amyloidosis AL type. © Springer Nature Switzerland AG 2024. All rights reserved.