Abstract
Cardiac involvement is the main determinant of prognosis in immunoglobulin light-chain (AL) amyloidosis, but may be under-recognized in patients with multiple myeloma (MM), particularly when classic electrocardiographic or cardiac magnetic resonance features are absent. Early recognition therefore relies on integration of cardiac biomarkers and multimodality imaging when tissue biopsy or scintigraphic subtyping is impractical.
A 74-year-old man with immunoglobulin A lambda MM and immune thrombocytopenic purpura developed progressive heart failure with preserved ejection fraction (HFpEF), increased left ventricular (LV) wall thickness, and markedly elevated N-terminal pro-B-type natriuretic peptide (NT-proBNP; 7164 pg/mL). Serial electrocardiograms did not demonstrate classic low voltage. Cardiac magnetic resonance revealed diffuse non-ischaemic late gadolinium enhancement consistent with an infiltrative cardiomyopathy. Bone-avid scintigraphy was non-contributory in the context of MM. A working diagnosis of highly likely AL cardiac amyloidosis was established. Clone-directed therapy (two cycles of bortezomib-cyclophosphamide-dexamethasone followed by three ongoing cycles of carfilzomib-lenalidomide-dexamethasone), together with volume-guided heart failure management, resulted in biochemical improvement (NT-proBNP 3431 pg/mL), although structural cardiac abnormalities persisted on follow-up imaging.