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Anti-Sa cold agglutinin of IgA class requiring plasma-exchange therapy as early manifestation of multiple myeloma

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Summary

The case of a patient (Col) with multiple myeloma presenting as chronic cold agglutinin (CA) syndrome is reported. The CA (Col) was a monoclonal IgA/k paraprotein which recognizes an antigen fully expressed in adult and newborn erythrocytes, sialidase sensitive and partially resistant to proteases. Hemaggluti-nation-inhibition studies showed that immunodominantN-acetylneuraminic acid bound α2 → 3 to O-glycans of glycophorins represents the CA(Col) epitope. These serological and biochemical findings fit with the anti-Sa specificity, of which only two previous examples are known. The clinical manifestations of CA (Col) were characterized by marked acrocyanosis, generalized livedo reticularis, and incapacitating dyspnea, but only mild hemolysis. Plasma-exchange therapy was effective in quickly removing the CA and relieving the associated clinical manifestations, but such benefit was only temporary. This is the first reported example of anti-Sa CA of IgA isotype and the first case of IgA CA syndrome treated by plasma exchange.

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Correspondence to A. Pereira.

Additional information

This work was partially supported by grant FISS 90/0486 from the Ministerio de Sanidad y Consumo of Spain

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Pereira, A., Mazzara, R., Escoda, L. et al. Anti-Sa cold agglutinin of IgA class requiring plasma-exchange therapy as early manifestation of multiple myeloma. Ann Hematol 66, 315–318 (1993). https://doi.org/10.1007/BF01695974

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Key words

  • Monoclonal gammopathy
  • Plasmapheresis