ABSTRACT

Most therapies for primary amyloidosis are directed at the plasma cell clone responsible for the synthesis of the insoluble immunoglobulin light chain. Both studies demonstrated a significant survival benefit for melphalan-containing regimens with the melphalan-containing arms showing a median survival of 17 months and the colchicine-alone arm showing a median survival of only 12 months. Dexamethasone therapy orally should be considered a viable regimen for the management of AL. Recently, a regimen of oral melphalan and oral dexamethasone has been reported for patients who are not suited, by virtue of age or performance status, for stem cell transplant. The day-100 mortality for stem cell transplant was 12% but has fallen to 6% in the last calendar year. Patients who are eligible for a stem cell transplant do inherently better than similar patients who are not eligible for transplant, a reflection of the selection that occurs in accepting patients for transplant.