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Diseases

Genetic and Rare Diseases Information Center (GARD)

Cold agglutinin disease


Other Names for this Disease
  • Anemia, hemolytic, cold antibody
  • Cold antibody hemolytic anemia
  • Cold antibody disease
  • CAD
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Treatment

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How might cold agglutinin disease be treated?

The treatment of cold agglutinin disease depends on many factors including the severity of the condition, the signs and symptoms present in each person, and the underlying cause. For example, in those affected by secondary cold agglutinin disease, it is important to diagnose and treat the underlying condition which may include certain types of cancer; bacterial, viral, or parasitic infections; and/or other autoimmune disease. People with few symptoms and/or mild anemia may not require any specific treatment. These cases are often managed by simply avoiding exposure to the cold. [1][2]

In severe cases of hemolysis, medical interventions may be necessary. Rituximab (an antibody that selectively reduces specific types of immune cells) is effective in about 60% of cases of severe cold agglutinin disease. Medical researchers have found the response to rituximab is seen on average within 1 to 2 months of treatment and the effect of the treatment lasts for about 1 to 2 years. Rituximab may be used after a second and even a third relapse, however the success rate is less. Combined treatment with rituximab and fludarabine has resulted in higher response rates (76% of cases) and longer periods of remissions (on average, 6.5 years). However the combined treatment may include serious side effects so is presently only recommended when rituximab has not worked alone. Finally, plasmapheresis, which involves filtering blood to remove antibodies, may be useful in acute hemolytic crisis and before surgery requiring hypothermia, however its effect is only short term. It should be noted, removing the spleen is not recommended for cold agglutinin disease.  In addition, because severe cold agglutinin disease requires very high doses of corticosteroids (levels not considered safe), corticosteroid treatment is no longer a recommended treatment in severe cases.[1][2]

Several possible therapies have been reported in a few case reports to have successfully treated people who are not responding to the treatments listed above.[1][2] However more studies need to be performed before the safety and effectiveness of these therapies can be determined.  Click the link to view the therapies presently being studied in cold agglutinin clinical trials
Last updated: 5/26/2016

References
  1. Stanley L Schrier, MD; Wendell F Rosse, MD. Clinical features and treatment of autoimmune hemolytic anemia: Cold agglutinins. UpToDate. September 11, 2015; http://www.uptodate.com/contents/clinical-features-and-treatment-of-autoimmune-hemolytic-anemia-cold-agglutinins.
  2. Barcellini W. Current treatment strategies in autoimmune hemolytic disorders. Expert Rev Hematol. October 2015; 8(5):681-91. http://www.ncbi.nlm.nih.gov/pubmed/26343892.


GARD Video Tutorial

  • Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.

    Finding Treatment Information

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Cold agglutinin disease. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
Other Names for this Disease
  • Anemia, hemolytic, cold antibody
  • Cold antibody hemolytic anemia
  • Cold antibody disease
  • CAD
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.