Figure 1.
Algorithm for the management of severe AIHA. AIHA = autoimmune hemolytic anemia; ESA = erythropoiesis-stimulating agents; LMWH = low molecular weight heparin; IVIg = Intravenous immunoglobulins; TPE = Therapeutic plasma exchange. Lower level of evidence in AIHA (see text). * Typically, unstable hemoglobin <8g/dl and/or hemodynamic instability and/or transfusion interval <7 days. ℂ Underlying diseases including, but not limited to: hematological malignancies, infectious diseases, other auto-immune disease (SLE), primary immunodeficiencies (4). † Daily monitoring of hemoglobin and hemolysis parameters. Consider monitoring in ICU. ‡ IgA AIHA, Mixed AIHA; rare and sometimes aggressive forms, typically recommendations for wAIHA are followed, consultation of expertise center is indicated. ¥ High dose steroids may be effective in severe CAD cases. Start tapering once rituximab has been started and discontinue in <6 months. # Therapeutic plasma exchange in CAD should occur at 37°C (including extracorporeal circuit); exchange plasma for albumin and not donor plasma. ¶ i.e., mycophenolate mofetil, cyclophosphamide, cyclosporin, azathioprine, danazol.