Recent advances in the management of AL Amyloidosis

Summary Immunoglobulin light chain (AL) amyloidosis, the most common of the systemic amyloidosis, is characterized by the deposition of amyloid fibrils that derive from the aggregation of misfolded monoclonal immunoglobulin light chains. Amyloid fibrils disrupt tissue architecture and the pre‐fibril...

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Veröffentlicht in:British journal of haematology Jg. 172; H. 2; S. 170 - 186
Hauptverfasser: Kastritis, Efstathios, Dimopoulos, Meletios A.
Format: Journal Article
Sprache:Englisch
Veröffentlicht: England 01.01.2016
Schlagworte:
Amyloidosis - diagnosis
Amyloidosis - immunology
Amyloidosis - therapy
heart failure
Humans
Immunoglobulin Light Chains - metabolism
Immunoglobulin Light-chain Amyloidosis
immunoglobulins
light chains
mass spectrometry
Melphalan - therapeutic use
monoclonal antibodies
Myeloablative Agonists - therapeutic use
Plasma Cells - drug effects
Plasma Cells - pathology
Risk Assessment - methods
Stem Cell Transplantation - methods
Treatment Outcome
heart failure
mass spectrometry
light chains
immunoglobulins
monoclonal antibodies
ISSN:0007-1048, 1365-2141, 1365-2141
Online-Zugang:Volltext
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Zusammenfassung:Summary Immunoglobulin light chain (AL) amyloidosis, the most common of the systemic amyloidosis, is characterized by the deposition of amyloid fibrils that derive from the aggregation of misfolded monoclonal immunoglobulin light chains. Amyloid fibrils disrupt tissue architecture and the pre‐fibril oligomers are directly toxic to myocardiac cells, causing cardiac dysfunction. The lethal consequences of AL amyloidosis are due to the toxic product and not due to the malignant behaviour of the plasma cell clone; however, the characteristics of this clone are associated with long‐term prognosis. Early and accurate diagnosis is the key to effective management, but is challenging. Modern chemotherapy options (including autologous transplantation, bortezomib, lenalidomide) have improved the outcomes of patients at low or intermediate risk, but the prognosis of patients with severe cardiac dysfunction is still poor. Therapies targeting amyloid deposits and the amyloidogenic process are under investigation and offer promise for better future treatments.
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ISSN:0007-1048
1365-2141
1365-2141
DOI:10.1111/bjh.13805