What is the treatment for cold agglutinin disease?

In cold agglutinin disease, the underlying issue is the presence of autoantibodies that cause the red blood cells to agglutinate at lower temperatures, leading to hemolytic anemia. The effective treatment for this condition focuses on managing the autoimmune response and reducing the production of these cold-reactive antibodies.

Cyclophosphamide and azathioprine are immunosuppressive agents that can help to reduce the production of these harmful antibodies. They work by inhibiting cell division and modulating the immune response, providing symptomatic relief and improving red blood cell counts by lessening the autoimmune attack on the erythrocytes.

While other options like rituximab are also used in some cases of cold agglutinin disease, cyclophosphamide and azathioprine specifically target the production of antibodies that cause the problem. Prednisone, a corticosteroid, can be used to reduce inflammation but may not be as effective as cyclophosphamide and azathioprine for chronic management. Plasmapheresis is typically reserved for acute cases to remove antibodies but does not address the underlying issue of antibody production in the long term.