What is the role of malignancy in the etiology of cold agglutinin disease?

Updated: Aug 28, 2018
  • Author: Salman Abdullah Aljubran, MD; Chief Editor: Michael A Kaliner, MD  more...
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Lymphoproliferative and autoimmune diseases, myeloma, Kaposi sarcoma, and angioimmunoblastic lymphoma may occasionally be associated with the production of cold agglutinins. An idea of associated disease distribution was provided by a study of 78 patients with persistent cold agglutinins. Among these patients, 31 had lymphoma, 13 had Waldenström macroglobulinemia, 6 had chronic lymphoid leukemia, and 28 had chronic idiopathic cold agglutinin disease. [11]

A case of cold agglutinin–induced hemolytic anemia has been described in association with an aggressive natural killer cell (NK-cell) leukemia. [33] Nonhematologic malignancies can occasionally be associated with a high-titer cold agglutinin–induced hemolytic anemia. [9, 34]

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