JACC. Case reports · 2026
Early Recovery of Cardiac Function After Hematologic Response in Systemic AL Amyloidosis.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Hari Aparna, Sidharthan Neeraj, Unni Manoj, Ahamed Hisham
Аннотация
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.
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.