How is cold agglutinin disease treated?

Updated: Aug 28, 2018
  • Author: Salman Abdullah Aljubran, MD; Chief Editor: Michael A Kaliner, MD  more...
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The treatment of cold agglutinin disease depends on the gravity of the clinical manifestations, as determined by the characteristics of the antibody and the presence of associated disease(s). Most patients with cold agglutinin disease can manage their disorder successfully just by wearing appropriate clothing and avoiding cold exposure. [47] Special protective clothing is sometimes necessary in extreme cases. Therapy is directed at serious symptoms and, in cases of secondary cold agglutinin disease, to the underlying disorder.

Keep in mind that the idiopathic variety of cold agglutinin disease is generally a benign disorder with prolonged survival. Spontaneous exacerbations and remissions in the course of the disease are to be expected. Acute postinfectious syndromes usually resolve spontaneously.

Anemia is generally mild. Only patients who have serious symptoms related to anemia or have a Raynaud-type syndrome that constitutes a threat to health or quality of life require active therapy.

Case studies report benefit from treatment with rituximab or the complement inhibitor eculizumab. Red blood cell (RBC) transfusion is indicated in severe, acute disease. Glucocorticoids are generally not useful in IgM-induced cold agglutinin disease but may occasionally work in selected patients. The presence of an associated malignancy requires specific therapy.

Cold agglutinin disease is so uncommon in children that no specific recommendations for therapy are available. Intravenous immunoglobulin (IVIG) was used successfully in an infant with IgA-associated autoimmune hemolytic anemia. [48]

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