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

Annals of Hematology volume 66, pages 315–318 (1993)Cite this article

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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References

  1. Angevine CD, Andersen BR, Barnett EV (1966) A cold agglutinin of the IgA class. J Immunol 96: 578–586

    Google Scholar 

  2. Bartholomeusz RCA, Forrest BD, Labrooy JT, et al. (1990) The serum polymeric IgA antibody response to typhoid vaccination; its relation to the intestinal IgA response. Immunology 60: 190–194

    Google Scholar 

  3. Crisp D, Pruzanski W (1982) B-cell neoplasms with homogeneous cold-reacting antibodies (cold agglutinins). Am J Med 72: 915–922

    Google Scholar 

  4. Curtis BR, Lamon J, Roelcke D, Chaplin H (1990) Life-threatening, antiglobulin test-negative, acute autoimmune hemolytic anemia due to non-complement activating IgGl κ cold antibody with Pra specificity. Transfusion 30: 838–843

    Google Scholar 

  5. Dahr W, Lichthardt D, Roelcke D (1981) Studies on the receptor sites of the monoclonal anti-Pr and -Sa cold agglutinins. Prot Biol Fluids 29: 365–368

    Google Scholar 

  6. Donovan DC, Collier VU, Baumann CG, Kickler TS (1983) Coombs-negative autoimmune hemolytic anemia associated with diffuse livedo reticularis. Md State Med J 32: 846–848

    Google Scholar 

  7. Garratty G, Petz LD, Brodsky I, Fudenberg HH (1973) An IgA high-titer cold agglutinin with an unusual blood group specificity within the Pr comlex. Vox Sang 25: 32–38

    Google Scholar 

  8. Klein HG, Balow JE, Dau PC, et al. (1986) Cold-agglutinin disease. J Clin Apheresis 2: 39–40

    Google Scholar 

  9. Mestecky J, Russell NW, Jackson S, Brown TA (1986) The human IgA system: a reassessment. Clin Immunol Immuno-pathol 40: 105–114

    Google Scholar 

  10. Pirofsky B, Roesner ER (1974) DDT test: a new method to differentiate IgM and IgG erythrocyte antibodies. Vox Sang 27: 480–488

    Google Scholar 

  11. Pruzanski W, Shumak KH (1977) Biologic activity of cold-reacting autoantibodies. N Engl J Med 297: 538–542 and 583–589

    Google Scholar 

  12. Rodenhuis S, Maas A, Hazenberg CAM, Das PC, Nieweg HO (1985) Inefficacy of plasma exchange in cold agglutinin hemolytic anemia —a case study. Vox Sang 49: 20–25

    Google Scholar 

  13. Roelcke D (1973) Specificity of IgA cold agglutinins: anti Pr1. Eur J Immunol 3: 206–212

    Google Scholar 

  14. Roelcke D (1989) Cold agglutination. Transfus Med Rev 2: 140–166

    Google Scholar 

  15. Roelcke D, Dorow W (1968) Besonderheiten der Reaktionsweise eines mit Plasmocytom-γA-Paraprotein identischen Kälteag-glutinins. Klin Wochenschr 46: 126–131

    Google Scholar 

  16. Roelcke D, Pruzanski W, Ebert W, et al. (1980) A new human monoclonal cold agglutinin Sa recognizing terminal N-acetyl-neuraminyl groups on the cell surface. Blood 55: 677–681

    Google Scholar 

  17. Roelcke D, Brossmer R, Ebert W (1981) Anti-Pr, -Gd and related cold agglutinins. Human monoclonal antibodies against neuraminyl groups. Prot Biol Fluids 29: 619–622

    Google Scholar 

  18. Roelcke D, Hengge U, Kirschfink M (1990) Neolacto(type-2 chain)-sialoautoantigens recognized by human cold agglutinins. Vox Sang 59: 235–239

    Google Scholar 

  19. Römer W, Rother U, Roelcke D (1980) Failure of IgA cold agglutinin to activate C. Immunobiology 157: 41–46

    Google Scholar 

  20. Rose VL, Kwaan HC (1985) Anti-Pr cold hemagglutination associated with livedo reticularis. Am J Hematol 19: 419–421

    Google Scholar 

  21. Sangster JM, Kenwright MG, Walker MP, Pembroke AC (1979) Anti blood group-M autoantibodies with livedo reticularis, Raynaud's phenomenon, and anemia. J Clin Pathol 32: 154–157

    Google Scholar 

  22. Somer T (1987) Rheology of paraproteinaemias and the plasma hyperviscosity syndrome. Baillieres Clin Haematol 1: 695–723

    Google Scholar 

  23. Suzuki S, Miura H, Hirakawa S, et al. (1985) A monoclonal IgM/k cold agglutinin bearing a new anti-Sa subspecificity. Acta Haematol Jpn 48: 1074–1082

    Google Scholar 

  24. Taft EG, Prop RP, Sullivan SA (1977) Plasma exchange for cold hemolytic anemia. Transfusion 17: 173–176

    Google Scholar 

  25. Tonthat H, Rochant H, Henry A, Leporrier M, Dreyfus B (1976) A new case of monoclonal IgA kappa cold agglutinin with anti-Pr1d specificity in a patient with persistent HB antigen cirrhosis. Vox Sang 30: 464–468

    Google Scholar 

  26. Uemura K, Roelcke D, Nagai Y, et al. (1984) The reactivities of human erythrocyte autoantibodies anti-Pr2, anti-Gd, -Fl, and -Sa with gangliosides in a chromatogram binding assay. Biochem J 219: 865–874

    Google Scholar 

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Authors and Affiliations

  1. Hemoterapia y Hemostasia, Hospital Clínico, Villarroel 170, 08036, Barcelona, Spain

    A. Pereira, R. Mazzara, L. Escoda & I. Alcorta

  2. Service of Clinical Hematology, Hospital Clínico, Barcelona, Spain

    B. Nomdedeu

  3. Institute for Immunology and Serology, University of Heidelberg, Heidelberg, Germany

    D. Roelcke

Authors
  1. A. Pereira
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  2. R. Mazzara
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  3. L. Escoda
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  4. I. Alcorta
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  5. B. Nomdedeu
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  6. D. Roelcke
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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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  • DOI: https://doi.org/10.1007/BF01695974

Key words

  • Monoclonal gammopathy
  • Plasmapheresis