Rituximab in cold agglutinin disease

Purpose. - Cold agglutinin disease is a chronic auto-immune hemolytic anemia related to a lymphoproliferative disorder with a degenerative potential and no codified treatment. This rare affection is related to the production of anti-erythrocytes immunoglobulins M. They are responsible of hemolytic crises sometimes severe and vascular acrosyndrom when submitted to cold temperature. Before rituximab, a monoclonal antibody targeted against the B lymphocyte CD20 antigen, no treatment was really efficient. Methods. - We present 5 patients who have been treated with 4 weekly rituximab perfusions, and then we proceed to a review of the literature concerning the other 23 similar cases. Results. - With a good tolerance, the treatment allowed a remission in all the cases (4 partial, I complete). Among the 23 observations published, the rate of answer was 21/23 (of which 14 gave completes). Conclusion. - Rituximab is an alternative treatment of cold agglutinin disease. (C) 2003 Editions scientifiques et medicales Elsevier SAS. Tous droits reserves.