Successful rituximab therapy in the treatment of refractory cold haemagglutinin disease with long-term disease control.

摘要

Cold agglutinin disease (CAD) is a rare disorder characterized by autoimmune haemolytic anaemia (AIHA), mediated by cold-reactive autoantibodies that bind to erythrocyte carbohydrate antigens, causing haemaggluti-nation and complement-mediated haemolysis. CAD is understood to be a B-cell disorder usually resistant to treatment with immunosuppressive therapy with sple-nectomy used, usually ineffectively, as a second-line therapy. Rituximab is a monoclonal antibody that is specific for the CD20 antigen, expressed on the surface of B lymphocytes and used in immune-mediated disorders.