Figure 3.
Recommended treatment of patients with SCD experiencing posttransfusion hemolysis. In such cases, transfusions should be stopped unless (indicated by asterisk) the patient has profound anemia (total <3 g/dL with shock or hyperlactemia), in which case rituximab is also indicated for patients with antibodies requiring transfusions. Symptomatic patients experiencing DHTR can be immediately treated with intravenous immunoglobulin (IVIg), adding erythropoietin (EPO) if the DHTR is also associated with reticulocytopenia. Prophylactic anticoagulation is administered to lower the risk of thrombosis associated with EPO administration. Supportive care is always indicated. If the patient has severity criteria (acute chest syndrome or acute pulmonary hypertension, stroke or organ failures), additional treatment with eculizumab can be effective.