ABSTRACT
The prognosis of patients with systemic AL is poor, with a median survival of 12–18 months and the main prognostic determinant is amyloid heart involvement. Therapy of AL is aimed at suppressing the plasma cell clone producing the amyloidogenic light-chain. The most effective treatment so far is autologous stem cell transplantation (ASCT), which can induce complete remissions in more than 50 percent of patients who can undergo this procedure. The patients in whom ASCT is contraindicated are those in greatest need of prompt care. They are usually treated with oral melphalan plus prednisone. However, response rate to this regimen is unsatisfactory and time to response can be unaffordably long. Time to response to high-dose dexamethasone (HD-Dex) is shorter (median 4 months), but response rate to HD-Dex is only 35 percent.
