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Incidence and natural history of primary systemic amyloidosis in Olmsted
County, Minnesota, 1950 through 1989 [see comments]
RA Kyle, A Linos, CM Beard, RP Linke, MA Gertz, WM O'Fallon and LT Kurland
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN
55905.
No reports of the incidence rates for primary systemic amyloidosis (AL)
have come to our attention. Records of all residents of Olmstead County,
Minnesota, with a diagnosis of amyloidosis were obtained from the Mayo
Clinic and its affiliated hospitals, as well as other medical groups that
might have seen local patients for the period January 1, 1950 to December
31, 1989. Twenty-one patients fulfilled the criteria for the diagnosis of
AL. The median age was 73.5 years, and 62% were men. In all but one patient
the diagnosis was made ante mortem. The clinical data of the 21 patients
were similar to those referral patients with AL seen at Mayo Clinic.
Immunohistochemical stains were positive for monoclonal light chains in the
amyloid deposits in 15 of the 21 cases. In six cases, tissue was not
available for immunohistochemical studies. Three of the six patients
without immunohistochemical stains had a free monoclonal lambda light chain
in the urine, and the other three had a monoclonal serum protein.
Immunoelectrophoresis/immunofixation detected a monoclonal (M)-protein in
the serum of 16 of 17 patients tested. A monoclonal light chain was found
in the urine of 10 of 15 patients. The overall sex- and age- adjusted rate
per million person-years was 6.1 from 1950 to 1969 and 10.5 from 1970 to
1989. The similarity of these rates suggests no significant increase over
time.
Volume 79,
Issue 7,
pp. 1817-1822,
04/01/1992
Copyright © 1992 by The American Society of Hematology
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