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Case Reports in Hematology
Volume 2019, Article ID 1610632, 6 pages
Case Report

Development of Multiple Myeloma of the IgA Type in a Patient with Cold Agglutinin Disease: Transformation or Coincidence?

1Section of Hematology, Department of Medicine, Haukeland University Hospital, Bergen, Norway
2Department of Pathology, Akershus University Hospital, Lørenskog, Norway
3Department of Research and Innovation, Haugesund Hospital, Haugesund, Norway

Correspondence should be addressed to Sigbjørn Berentsen; on.ttenguah@nestnereb.nrojbgis

Received 31 October 2018; Accepted 12 February 2019; Published 4 March 2019

Academic Editor: Masayuki Nagasawa

Copyright © 2019 Øystein Sefland et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia and a distinct, clonal bone marrow lymphoproliferative disorder, characterized in most cases by a monoclonal IgMκ serum protein. We describe a CAD patient presenting with a monoclonal immunoglobulin of the IgAλ class. For years, she remained asymptomatic apart from the hemolytic anemia until eventually she developed multiple myeloma (MM) of the IgAλ phenotype. Prior to the development of MM, her hemolytic anemia responded to rituximab monotherapy. After she was diagnosed with MM, both conditions responded well to bortezomib-based therapy. We performed further investigations to determine whether her MM represented a progression/transformation of CAD or an independent disease. Flow cytometry and biopsy findings convincingly confirmed two unrelated clonal B-cell disorders. On this background, we also discuss previously published reports on cold agglutinin activity in patients with IgA gammopathy. In conclusion, cold agglutinins of the IgA class do probably not result in CAD. If a monoclonal immunoglobulin other than IgMκ is found in a patient with CAD, the coexistence of two unrelated B-cell clones should be suspected.