Survival in systemic AL amyloidosis. (A) Updated survival of the first MDex trial (n = 46) is shown. Median progression-free and overall survivals are 3.8 and 5.1 years, respectively. Data from Palladini et al¹⁰⁰  with permission. Treatment with oral melphalan plus dexamethasone produces long-term remissions in AL amyloidosis. (B) The phase 3 comparison of MDex (blue curve) and SCT (red curve) shows improved survival for MDex and dramatic early mortality for SCT (n = 50 in each arm). Reprinted from Jaccard et al⁵³  with permission. (C) Survival by response is shown for AL cardiac patients not eligible for SCT and treated with MDex (n = 37). Variables that negatively influenced survival included baseline troponin I level more than 0.12 ng/mL, interventricular septal thickness more than 1.4 cm, and male sex. Responders had a median survival of 22 months.⁵⁵  (D) The survival of AL SCT patients by cardiac stage is shown. Data from Dispenzieri et al⁶³  with permission. Staging is defined by NT-proBNP and troponin T thresholds of 332 pg/mL and 0.035 ng/mL: stage I, both NT-proBNP and troponin T under threshold; stage II, either over threshold; and stage III, both equal to or over threshold. (E) Updated survival is shown for AL patients undergoing SCT and then receiving adjuvant thalidomide and dexamethasone therapy if clonal plasma cell disease persisted (n = 45).²¹  At median follow-up of 52 months, 69% of patients survive.