Cold-agglutinin-induced autoimmune hemolytic anemia (AIHA) is difficult to treat. It is also called cold agglutinin disease. Cold agglutinin disease is a group of disorders that are characterized by antierythrocyte autoantibodies that bind red blood cells at cold temperatures (< 37° C), causing hemagglutination. The antibodies are usually of the IgM type but may also be of the immunoglobulin G (IgG) or immunoglobulin A (IgA) type. Symptoms include Raynaud’s phenomena (vasospasm of peripheral arterioles) and acrocyanosis (intra-arteriolar agglutination of red blood cells in the fingers, feet, nose and earlobes, causing numbness, stiffness and pain in these areas). A phase II study of Rituxan is currenlty ongoing in cryoglobulinemia, which is a related disorder.
Rituximab has been used for the treatment of chronic cold agglutinin disease in several case studies and in 1 small trial was 375 mg/m2 weekly for 4 doses. However until a randomized study can be conducted, it remains an investigational option. A 2007 review concluded: “The preliminary results are encouraging, but further studies are required in order to allow firm conclusions.”
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