AL Amyloidosis for the Cardiologist and Oncologist: Epidemiology, Diagnosis, and Management

Ronald M Witteles, Michaela Liedtke, Ronald M Witteles, Michaela Liedtke

Abstract

AL amyloidosis results from clonal production of immunoglobulin light chains, most commonly arising from a clonal plasma cell disorder. Once considered a nearly uniformly fatal disease, prognosis has improved markedly over the past 15 years, predominantly because of advances in light chain suppressive therapies. Cardiac deposition of amyloid fibrils is common, and the severity of cardiac involvement remains the primary driver of prognosis. Improvements in chemotherapy/immunotherapy have prompted a reassessment of the role of advanced cardiac therapies previously considered contraindicated in most patients, including the role of implantable cardioverter-defibrillators and cardiac transplantation. This state-of-the-art review highlights the current state of the field, including diagnosis, prognosis, and hematologic- and cardiac-specific therapies.

Keywords: AL amyloidosis; ASCT, autologous stem cell transplantation; BNP, B-type natriuretic peptide; CyBorD, cyclophosphamide, bortezomib, and dexamethasone; FLC, free light chain; ICD, implantable cardioverter-defibrillator; MGUS, monoclonal gammopathy of undetermined significance; NT-proBNP, N-terminal pro–B-type natriuretic peptide; SAP, serum amyloid P; SPIE, serum protein electrophoresis with immunofixation; UPIE, urine protein electrophoresis with immunofixation; amyloidosis; diagnosis; drug therapy; heart failure; imaging; treatment.

© 2019 The Authors.

Figures

Graphical abstract
Graphical abstract
Figure 1
Figure 1
Cardiac Amyloidosis Pathology (Left) Hematoxylin-eosin stain of an endomyocardial biopsy sample demonstrating the characteristic pale eosinophilic amyloid deposits surrounding each cardiomyocyte. (Right) Trichome staining highlights amyloid deposits in blue. Images courtesy of Gerald Berry, MD, Stanford Amyloid Center.
Central Illustration
Central Illustration
Algorithm for Evaluation for Suspected Cardiac Amyloidosis If SPIE, UPIE, or FLC ratio are abnormal, it is reasonable to proceed with fat pad or bone marrow biopsy or to proceed directly to endomyocardial biopsy depending on institutional preference. However, if fat pad and/or bone marrow biopsy do not reveal amyloid deposits, endomyocardial biopsy should be pursued. H:CL = heart to contralateral lung ratio; other abbreviations in Table 1.

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