When Hematologic Response Does Not Translate to Cardiac Recovery in AL Amyloidosis

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.

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.

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.

Early hematologic response does not ensure cardiac stabilization in advanced cardiac AL amyloidosis.