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

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.