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.