A patient with a demyelinating sensory motor polyneuropathy secondary to IgM paraproteinaemia is reported. The paraprotein binds to the gangliosides GD1b, GT1b, GQ1b and GD3, all of which contain disialosyl groups with the sequence NeuAcα2-8NeuAcα2-3Gal. The paraprotein also acts as a cold agglutinin recognising the sialic-acid-dependent Pr(1d) antigenic determinant of the red cell membrane glycophorins. In this and in similar cases that have been reported, the coexistence of anti-Pr cold agglutinins and peripheral neuropathy suggest that they might be the causative agents of the disease.
Cold agglutinins with the rare anti-Pr specificity were identified in an adult patient with fresh varicella infection. The antibody was of the IgM x type with subspecificity anti-Pr3d and caused a haemolytic episode in the patient. In the only previously reported case in which cold agglutinins were associated with varicella infection, the antibody specificity was also anti-Pr.
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