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Autosomal dominant cerebrovascular amyloidosis: Properties of peripheral blood lymphocytes

Selected Properties of Peripheral blood lymphocytes (PBLs) from five ambulatory affected individuals of a kindred with autosomal dominant cerebrovascular amyloidosis were studied. The percentage of PBLs bearing surface membrane immunoglobulin (SmIg + cells) was increased in the patient group (30 ± 3% versus 20 ± 2%; p < 0.05). The Percentage of PBLs forming early and total E‐rosettes was comparable in patient and control groups. Mitogenic response to concanavalin A (Con A) was suggestively reduced in the patient group, measured both by total 3Hthymidine incorporation and by comparison of stimulation indices. Mitogenic response to phytohemagglutinin and pokeweed was comparable in the two groups. Capping of Con A by PBLs was significantly reduced in the patient group compared with the controls (13 ± 1% versus 26 ± 2%; p < 0.01). The findings of reduced Con A response and increased SmIg+ cells support the hypothesis that immune dysfunction contributes to the development of amyloidosis. The reduced capping suggests altered membrane properties in this autosomal dominant disorder.