Anti-CD20 monoclonal antibody for the treatment of severe, immune-mediated, pure red cell aplasia and hemolytic anemia

Immune-mediated, acquired pure red cell aplasia (PRCA) is a rare disorder f requently associated with other autoimmune phenomena. Conventional immunosu ppressive treatment is often unsatisfactory. Rituximab is a monoclonal anti body against the CD20 antigen, highly effective for in vivo B-cell depletio n. An 18-month-old girl with both severe PRCA and autoimmune hemolytic anem ia, refractory to immunosuppressive treatment, received 2 doses of rituxima b, 375 mg/m(2) per week, The drug was well tolerated. After anti-CD20 thera py, substitutive treatment with intravenous immunoglobulin was started. The treatment resulted in marked depletion of B cells; a striking rise in reti culocyte count ensued, with increasing hemoglobin levels, finally leading t o transfusion independence. The child is now 5 months off-therapy, with nor mal hemoglobin and reticulocyte levels. This case suggests a role of anti-C MD monoclonal antibody for treatment of patients with antibody-mediated hem atologic disorders.