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Resolution of heart failure in patients with AL amyloidosis
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 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.