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Management of refractory autoimmune hemolytic anemia via allogeneic stem cell transplantation

Bone Marrow Transplantation volume 51, pages 1504–1506 (2016)Cite this article

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Waldenström’s macroglobulinemia (WM) represents a subset of lymphoplasmacytic lymphomas in which clonally related lymphoplasmacytic cells secrete a monoclonal IgM Ab.1 Overproduced IgMs can act as cold agglutinins in WM. Upon exposure to cooler temperatures in the periphery, they cause anemia via binding to the erythrocyte Ii-antigen group and classical complement cascade initiation.2 Treatment of cold-agglutinin-mediated autoimmune hemolytic anemia (AIHA) in WM typically targets the pathogenic B-cell clone1, 2, 3, 4 or the classical complement cascade.2, 5, 6 Plasmapheresis rapidly lowers IgM concentrations for immediate disease control.1 We report for the first time a case of WM with presumed cold-agglutinin-mediated AIHA refractory to typical therapy, ultimately requiring hematopoietic stem cell transplantation, specifically for anemia control.

A 52-year-old female with a history of hypertension, Raynaud’s syndrome, and familial WM (low level myd88 c.794T>C (p.Leu265Pro) mutation present; father and grandfather have WM, and brother has WM with related angioedema) presented to the emergency room with worsening livedo reticularis and fatigue.

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Authors and Affiliations

  1. Harvard Medical School, Boston, MA, USA

    L Rotenstein

  2. Department of Internal Medicine, Brigham and Women’s Hospital, Boston, MA, USA

    A Nathan

  3. Department of Medical Oncology, Dana Farber Cancer Institute, Boston, MA, USA

    I Ghobrial & J Antin

  4. Division of Hematology, Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA

    A Parnes

Authors
  1. L Rotenstein
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  2. A Nathan
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  3. I Ghobrial
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Correspondence to A Parnes.

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Competing interests

AP has received compensation as a consultant for Alexion, the manufacturer of eculizumab. The remaining authors declare no conflict of interest.

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Rotenstein, L., Nathan, A., Ghobrial, I. et al. Management of refractory autoimmune hemolytic anemia via allogeneic stem cell transplantation. Bone Marrow Transplant 51, 1504–1506 (2016). https://doi.org/10.1038/bmt.2016.152

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