Long-term survival (10 years or more) in 30 patients with primary amyloidosis

The median survival in primary systemic (AL) amyloidosis is less than 18 mo nths. No published series of patients with AL amyloidosis have reported sur vival of more than 10 years. The records of all Mayo Clinic patients with a diagnosis of AL amyloidosis between January 1, 1966 and March 1, 1987 were reviewed. Patients with secondary amyloidosis, familial amyloidosis, senil e systemic amyloidosis, and localized amyloidosis were excluded. During the 21 years of the study, 841 patients with AL amyloidosis were seen. Of thes e, 29 were excluded because the diagnosis was made at autopsy, and 2 others were excluded because no follow-up data were available. Actuarial survival for the 810 patients was 51% at 1 year, 16% at 5 years, and 4.7% at 10 yea rs. Thirty patients survived for 10 years or more after the histologic diag nosis of AL amyloidosis; all received alkylating-agent therapy. In 14 patie nts, the monoclonal protein disappeared from the serum or urine. Of 10 pati ents with nephrotic syndrome, 4 had an objective response. Congestive heart failure, older age, creatinine value of 2 mg/dL or more, bone marrow plasm a cell value of 20% or more, platelet count of 500 x 10(9)/L or less, and t he presence of peripheral neuropathy were underrepresented in the 10-year s urvivors and are unfavorable prognostic features. Five percent of patients with AL amyloidosis survived for 10 years or more. (C) 1999 by The American Society of Hematology.