Cold agglutinin disease: Role of rituximab

Cold agglutinin disease is an acquired autoimmune hemolytic anemia, induced by auto-antibodies that, at a cold temperature, bind to the surface of red blood cells and induce lysis. Prevalence is 16 per million inhabitants. This chronic hemolysis of moderate severity affects elderly people and causes anemia, jaundice and acrocyanosis. Symptoms are worsened by exposure to cold temperatures and fever. Diagnosis is made from clinical features, biology, direct Coombs'test and discovery of cold agglutinin in vitro. Rituximab seems to be an efficient treatment with low toxicity, as observed in our case report. Thus, we report the case of one patient successj fully treated with 40 rituximab infusions from June 2001 to December 2008.