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 less than 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 3H-thymidine 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 less than 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.
D000328:Adult; D000686:Amyloidosis / Q000235:genetics*; D002462:Cell Membrane / Q000032:analysis; D002561:Cerebrovascular Disorders / Q000235:genetics* / Q000276:immunology; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D005799:Genes, Dominant; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D007152:Immunologic Capping; D008213:Lymphocyte Activation; D008214:Lymphocytes / Q000276:immunology*; D008934:Mitogens / Q000494:pharmacology; D012397:Rosette Formation
D007136:Immunoglobulins; D008934:Mitogens