Key result
CyBorD yields rapid cardiac functional recovery, improving LVEF by ~18 points in AL amyloidosis.
Why the study?
Cardiac functional recovery in systemic AL amyloidosis typically lags behind hematologic response by months to years, and early recovery patterns are not well characterized.
Case Report (n=1)
No
Early functional cardiac recovery on echocardiography (GLS, LVEF) can rapidly accompany deep hematologic response in AL amyloidosis, suggesting value in routine imaging surveillance beyond NT-proBNP alone.
Suggests value of serial GLS monitoring in AL amyloidosis; hypothesis-generating and requires prospective validation.
BACKGROUND: Cardiac involvement determines prognosis in systemic AL amyloidosis, and functional cardiac recovery typically lags hematologic response by months to years. CASE SUMMARY: A 40-year-old woman with biopsy-proven systemic AL amyloidosis and cardiac, renal, and hepatic involvement received cyclophosphamide, bortezomib, and dexamethasone, achieving rapid deep hematologic response, later consistent with complete response. Left ventricular ejection fraction improved from 40% to 58% and global longitudinal strain (GLS) from -9.2% to -12.1% within 4 months. At 1 year, GLS was -15.8%, and N-terminal pro B-type natriuretic peptide (NT-proBNP) fell from 2,397 pg/mL to 65 pg/mL, with hepatic and renal responses. DISCUSSION: Cardiac recovery in AL amyloidosis is usually delayed. The rapid improvement in left ventricular ejection fraction, GLS, and NT-proBNP in this case suggests relief from light chain-mediated injury with possible evolving amyloid regression. TAKE-HOME MESSAGES: Early cardiac functional recovery may accompany deep hematologic response in AL amyloidosis. Serial echocardiography, including GLS, complements NT-proBNP in tracking cardiac response beyond hematologic remission alone.
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Hari et al. (2026) conducted a case report in Systemic AL amyloidosis (n=1). CyBorD (cyclophosphamide, bortezomib, dexamethasone) was evaluated on Cardiac functional recovery (LVEF, GLS, and NT-proBNP). CyBorD therapy led to rapid cardiac functional recovery, improving LVEF from 40% to 58% and GLS from -9.2% to -12.1% within 4 months in a patient with systemic AL amyloidosis.
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