Key result
Dara-CyBorD achieves ~95% light-chain reduction but fails to prevent fatal cardiac amyloidosis progression.
Why the study?
Hematologic response in cardiac AL amyloidosis does not always lead to cardiac recovery, especially in advanced disease stages.
Case Report (n=1)
No
Early hematologic response to contemporary therapy does not guarantee cardiac stabilization in advanced AL amyloidosis, and patients remain at high risk of rapid hemodynamic collapse and sudden death.
BACKGROUND: Cardiac light-chain (AL) amyloidosis carries high early mortality due to rapid hemodynamic deterioration. Hematologic response does not always translate into cardiac recovery, particularly in advanced disease. CASE SUMMARY: A 66-year-old woman presented with dyspnea and chronic edema. Electrocardiography showed low QRS voltage, and echocardiography revealed severe concentric thickening with apical sparing on strain imaging. Free λ light chains were markedly elevated, and bone marrow biopsy confirmed plasma cell myeloma. Cardiac magnetic resonance imaging supported infiltrative cardiomyopathy. Daratumumab-based chemotherapy resulted in substantial reduction in free λ levels. Despite hematologic response, she remained hypotensive with persistent right ventricular dysfunction and intolerant to guideline-directed therapy. Five months after presentation, she died suddenly at home. DISCUSSION: This case illustrates the dissociation between biochemical improvement and cardiac stabilization in advanced AL amyloidosis and highlights the persistent risk of hemodynamic collapse and sudden death. TAKE-HOME MESSAGE: Early hematologic response does not ensure cardiac stabilization in advanced cardiac AL amyloidosis.
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Futela et al. (2026) conducted a case report in Cardiac light-chain (AL) amyloidosis and plasma cell myeloma (n=1). Daratumumab-cyclophosphamide-bortezomib-dexamethasone (Dara-CyBorD) was evaluated on Clinical outcome.
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