Which hematologic diseases are associated with cold agglutinin disease?

Updated: Aug 28, 2018
  • Author: Salman Abdullah Aljubran, MD; Chief Editor: Michael A Kaliner, MD  more...
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A review of Mayo Clinic patients with cold agglutinin disease identified underlying hematologic diseases in 69 of 89 patients (76%). Monoclonal gammopathy of undetermined significance (MGUS) constituted 42 cases. The other hematologic disorders were lymphoproliferative disorders: macroglobulinemia, chronic lymphocytic lymphoma, other lymphomas (including low-grade B-cell and diffuse large B-cell lymphoma), and cutaneous T-cell lymphoma. [1]

Monoclonal cold agglutinin IgM antibodies found in patients with lymphoma are the product of the abnormal clone. Progression of an idiopathic cold agglutinin disease to malignant lymphoma may occur in some cases; thus, affected patients require close, long-term follow-up, with obvious therapeutic implications. [27, 13] One study of 86 patients in Norway showed clonal light-chain predominance in 90% of patients, evidence of lymphoplasmacytic lymphoma in 50% of patients, and lymphoma of any type in 76% of patients overall. [1]

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