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Blood, Vol. 93 No. 3 (February 1), 1999:
pp. 1062-1066
Long-Term Survival (10 Years or More) in 30 Patients With Primary
Amyloidosis
Robert A. Kyle,
Morie A. Gertz,
Philip R. Greipp,
Thomas E. Witzig,
John A. Lust,
Martha Q. Lacy, and
Terry M. Therneau
From the Division of Hematology and Internal Medicine and the Section
of Biostatistics, Mayo Clinic and Mayo Foundation, Rochester, MN.
The median survival in primary systemic (AL) amyloidosis is less
than 18 months. No published series of patients with AL amyloidosis have reported survival 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, senile systemic amyloidosis, and
localized amyloidosis were excluded. During the 21 years of the study,
841 patients with AL amyloidosis were seen. Of these, 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 years. Thirty patients survived for 10 years or more after the
histologic diagnosis of AL amyloidosis; all received alkylating-agent
therapy. In 14 patients, the monoclonal protein disappeared from the
serum or urine. Of 10 patients with nephrotic syndrome, 4 had an
objective response. Congestive heart failure, older age, creatinine
value of 2 mg/dL or more, bone marrow plasma cell value of 20% or
more, platelet count of 500 × 109/L or less, and the
presence of peripheral neuropathy were underrepresented in the 10-year
survivors and are unfavorable prognostic features. Five percent
of patients with AL amyloidosis survived for 10 years or more.
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