Primary amyloidosis (AL): Referral center vs multicenter patient populations -clinical implications

AL is caused by deposition in target tissues of amyloid fibrils constituted by immunoglobulin light chains produced by a usually modest bone marrow plasma cell clone. Prognosis and therapeutic strategy depend on the predominant organ involved. A median survival of 17 months was reported by the Mayo Clinic referral Center, while the multicenter Italian AL Study Group, involving 80 clinical centers covering all Italian regions, reported a 40 months median survival. Elucidation of the causes of such discrepancy may lead to & better understanding of the natural history of the disease and at improving the patient care. All patients (pts) initially diagnosed with systemic AL from June 1,1988, through June 30, 1998, at Mayo Clinic or in any of the Italian centers were included in this study. Patients were treated with a melphalan-based chemotherapy. Analysis of variance was used to compare: the two groups, and log-rank test to compare survivals. The Mayo Clinic group was composed of 281 pts, 189 (67%) male and 92 (33%) female with a median age of 67 years (39-89). The Italian group was composed of 209 pts, 119 (57%) male and 90 (43%) female with a median age of 62 years (29-85). The difference in gender and age between the two groups was statistically significant (p=0.02 and