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Resolution of Heart Failure in Patients with AL Amyloidosis

Simon Dubrey, MD; Lisa Mendes, MD; Martha Skinner, MD; and Rodney H. Falk, MD
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From Boston University School of Medicine, Boston, Massachusetts. Acknowledgments: The authors thank J.C. Nelson, MD (Westchester County Medical Center, Valhalla, New York) for detailed information about patient 1. Grant Support: In part by the Sue Sellors Finley Cardiac Amyloid Research Fund. Requests for Reprints: Rodney H. Falk, MD, Section of Cardiology, Boston Medical Center, 818 Harrison Avenue, Boston, MA 02118. Current Author Addresses: Drs. Dubrey, Mendes, and Falk: Division of Cardiology, Boston University School of Medicine, Boston, MA 02118.

Copyright ©2004 by the American College of Physicians

Ann Intern Med. 1996;125(6):481-484. doi:10.7326/0003-4819-125-6-199609150-00009
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Background: Patients with AL amyloidosis and congestive heart failure have a very poor prognosis. To date, the recovery of these patients has not been described in detail.

Objective: To determine the frequency and characteristics of regression of disease in patients with congestive heart failure due to AL amyloidosis.

Design: Review of patients with systemic AL amyloidosis.

Setting: An international referral center for amyloidosis in the United States.

Patients: 140 patients with congestive heart failure due to AL amyloidosis who were seen between 1983 and 1994.

Measurements: Functional status, Doppler echocardiography, and objective measurements of disease activity.

Results: 3 of 140 patients (2.1%) had marked resolution of congestive heart failure and evidence for remission of disease activity. All 3 had been treated with melphalan.

Conclusions: Melphalan appears to have had a favorable effect in 3 patients with AL amyloidosis and heart failure. The abolition of light chains that was seen in these 3 patients suggests that light-chain toxicity may play a role in the genesis of heart failure in patients with AL amyloidosis.





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