TY  - JOUR
SP  - 152
SN  - 1874-1754
AV  - public
JF  - International Journal of Cardiology
KW  - Science & Technology
KW  -  Life Sciences & Biomedicine
KW  -  Cardiac & Cardiovascular Systems
KW  -  Cardiovascular System & Cardiology
KW  -  AL amyloidosis
KW  -  Heart
KW  -  Therapies
KW  -  STEM-CELL TRANSPLANTATION
KW  -  HIGH-DOSE MELPHALAN
KW  -  RISK-ADAPTED MELPHALAN
KW  -  AL AMYLOIDOSIS
KW  -  CARDIOVERTER-DEFIBRILLATOR
KW  -  HEART-TRANSPLANTATION
KW  -  P COMPONENT
KW  -  PHARMACOLOGICAL DEPLETION
KW  -  EXTRACELLULAR AGGREGATION
KW  -  NATRIURETIC PEPTIDE
PB  - ELSEVIER IRELAND LTD
N1  - This version is the author accepted manuscript. For information on re-use, please refer to the publisher?s terms and conditions.
Y1  - 2018/11/15/
TI  - Therapies for cardiac light chain amyloidosis: An update
N2  - Light-chain (AL) amyloidosis is the most common type of systemic amyloidosis, affecting around 10 people per million per year. This serious disorder is characterized by the presence of a clone of bone marrow plasma cells that produces monoclonal light chains (LCs) of the ? or predominantly ? type. These amyloidogenic LCs undergo extracellular misfolding and aggregation into proteotoxic soluble oligomers and amyloid fibrils that deposit within tissues. The lethal consequences of AL amyloidosis are due to the toxic products (the LCs) and not to the malignant behaviour of the plasma cell clone. Almost 80% of patients with AL amyloidosis have some degree of cardiac involvement, manifesting as heart failure (HF), and carrying a particularly poor prognosis.

The past decade has seen major advances in the treatment of AL amyloidosis, and a rapidly fatal disease has become a treatable and possibly curable condition. The number of therapeutic options is rapidly expanding, offering hope to address currently unmet needs (most notably, the treatment of frail patients). The treatment of AL amyloidosis consists in a combination of agents targeting multiple steps of the amyloid cascade, associated with effective HF management, and there is ground for hope for dramatically improving the outcome in the near future.

In the present review we will summarize our current knowledge on therapy for cardiac AL amyloidosis, targeting clinical cardiologists involved in the care of this serious disorder.
EP  - 160
A1  - Aimo, A
A1  - Buda, G
A1  - Fontana, M
A1  - Barison, A
A1  - Vergaro, G
A1  - Emdin, M
A1  - Merlini, G
VL  - 271
UR  - https://doi.org/10.1016/j.ijcard.2018.05.018
ID  - discovery10073353
ER  -