ABSTRACT

Treatments for primary amyloidosis (AL) are based on reducing the supply of the circulating amyloidogenic light chain by targeting the plasma cell clone responsible for its synthesis. High-dose melphalan followed by autologous stem cell transplantation (ASCT) induces complete remissions in a considerable proportion of eligible patients. Current effective treatments in patients not eligible for ASCT include oral melphalan associated with prednisone or dexamethasone, or high-dose dexamethasone alone. Still, a significant proportion of AL patients do not respond to first-line therapy. Hematologic response was defined as the decrease of the monoclonal component (MC) by at least 50%. Complete remission consisted in the disappearance of the MC from both serum and urine documented by high-resolution agarose-gel immunofixation electrophoresis. The association of dexamethasone and thalidomide provided a 35% hematologic response rate and functional improvement of the organs involved in 20% of patients.