Key result
Dara-CyBorD achieves complete remission and potential myocardial reversal in ~5 months for AL cardiac amyloidosis.
Why the study?
Light-chain cardiac amyloidosis is often overlooked and misdiagnosed as simple diastolic dysfunction, leading to worse outcomes if diagnosis is delayed.
Case Report (n=1)
Prompt diagnosis and tailored hematologic treatment with Dara-CyBorD can lead to complete remission and stabilization of myocardial dysfunction in light-chain cardiac amyloidosis.
Hypothesis-generating for Dara-CyBorD in light-chain cardiac amyloidosis; should not change practice without prospective trials.
BACKGROUND: Light-chain cardiac amyloidosis (AL-CA) is often overlooked and misdiagnosed as simple diastolic dysfunction, which can lead to worse outcomes if an accurate diagnosis is delayed. CASE SUMMARY: This case highlights the importance of utilizing a multidisciplinary team approach and multimodality imaging, such as cardiac magnetic resonance and endomyocardial biopsy, in the prompt diagnosis and treatment of AL-CA with a tailored hematologic treatment regimen in a 70-year-old male. Patient achieved complete remission with Dara-CyBorD (daratumumab, cyclophosphamide, bortezomib, and dexamethasone) therapy in 5 months, demonstrating stabilization and potential reversal of myocardial dysfunction. DISCUSSION: Of the many causes of heart failure with preserved ejection fraction, AL-CA can have an especially poor prognosis if diagnosis is delayed. It is crucial to be aware of the clinical presentation of AL-CA, the wide variety of diagnostic tools, multidisciplinary management, and intensive treatment options available, and importance of regular follow-up. TAKE-HOME MESSAGES: Recognizing the hallmark diagnostic features of AL-CA is crucial for differentiating it from other causes of diastolic dysfunction, particularly in the early work-up period. Timely diagnosis of AL-CA and assessment of the infiltrative burden are essential for selecting an individualized hematologic treatment course.
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Rahman et al. (2025) conducted a case report in Light-chain cardiac amyloidosis (AL-CA) (n=1). Dara-CyBorD (daratumumab, cyclophosphamide, bortezomib, and dexamethasone) was evaluated on Complete remission and stabilization/reversal of myocardial dysfunction. Dara-CyBorD therapy achieved complete remission and potential reversal of myocardial dysfunction in 5 months in a 70-year-old male with light-chain cardiac amyloidosis.
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