What is the role of chemotherapy in the treatment of cold agglutinin disease?

Updated: Aug 28, 2018
  • Author: Salman Abdullah Aljubran, MD; Chief Editor: Michael A Kaliner, MD  more...
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Chemotherapeutic agents should be used under appropriate circumstances, such as for an associated malignancy. However, the authors currently do not recommend the use of chemotherapeutic or immunosuppressive agents in the routine management of idiopathic cold agglutinin disease. Such agents are not usually needed in these cases; they provide little benefit, given the basic benign nature of the disease, and they pose high risk because of their potential long-term adverse effects on bone marrow stem cells, including the leukemogenic effects of alkylating agents.

When idiopathic cold agglutinin disease is sufficiently severe that one must weigh the need for therapy, such decisions should be made in close collaboration with patients and their families. Patients need to be fully informed about the adverse effects of chemotherapeutic or other agents used to treat monoclonal lymphoid populations. If they decide to proceed with therapy, the agents used must be selected with extreme caution.

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