Autosomal dominant cerebrovascular amyloidosis: Properties of peripheral blood lymphocytes
1980; Wiley; Volume: 7; Issue: 5 Linguagem: Inglês
10.1002/ana.410070508
ISSN1531-8249
AutoresKári Stéfansson, Jack P. Antei, Joël Oger, James Burns, AvertanoB. C. Noronha, Raymond P. Roos, Barry G.W. Arnason, Gunnar Guðmundsson,
Tópico(s)Renal Diseases and Glomerulopathies
ResumoAbstract 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 3 Hthymidine 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.
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