抗Pr2特異性を示したlow titer cold agglutinin disease

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  • Low-titer cold agglutinin disease with anti-Pr2 specificity.

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We report a case of low-titer cold agglutinin disease with ant-Pr2 specificity and ataxic polyneuropathy associated with IgMκ monoclonal gammopathy, that is the first report in Japan.<br>The cold agglutinin (CA) reacted with untreated OI and Oi cells, and untreated and proteasetreatd dog RBCs. Both protease and sialidase treatments of human RBCs abolished its reactivity, and these findings confirmed ant-Pr2 specificity. Agglutination with the anti-Pr2 CA was inhibitted by beaf brain gangliosides and GM3, and weakly by G1a, GD1b, GT1b and glycophorin. The anti-Pr2 could play a pathogenetic role not only in hemolytic anemia, but also in peripheral nerve demyelination in this case, because gangliosides GM3, GD1a, GD1b and GT1b are constituents of human peripheral nerve myelin.

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  • 日本輸血学会雑誌

    日本輸血学会雑誌 38 (1), 70-74, 1992

    一般社団法人 日本輸血・細胞治療学会

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