Severe cold hemagglutinin disease (CHD) successfully treated with rituximab

Autoimmune hemolytic anemia results from an IgM antibody (Ab)-red cell interaction and is referred to as cold hemagglutinin disease (CHD). CHD is most commonly associated with infectious or lymphoproliferative diseases but may also be coupled with malignancies or autoimmune or immunodeficiency syndromes. Cold agglutinin-mediated hemolysis occurs at low temperatures, may be severe, and is notoriously difficult to treat. CHD patients with infections often have a short clinical course, whereas those with lymphoma require therapy. Therapeutic maneuvers successful in patients with warm Ab-associated autoimmune hemolytic anemia such as corticosteroids, intravenous immunoglobulin G (IgG), and splenectomy are usually ineffective in CHD. ] In vitro studies have demonstrated that the antibody binds human C1q and induces complementdependent cytotoxicity, antibody-dependent, cell-mediated cytotoxicity, and apoptosis. 4] ere we report on 2 patients with severe CHD, who were both successfully treated with the chimeric anti-CD20 monoclonal Ab rituximab. The patients' disease course and response to rituximab are displayed in Figure Both patients were seronegative for HIV-1 and HIV-2, and other infections were ruled out by appropriate diagnostic procedures.