The patient should not receive an erythrocyte transfusion at this time. Erythrocyte transfusion in sickle cell disease (SCD) is appropriate only for specific indications, including stroke, symptomatic anemia, acute chest syndrome (ACS), surgical interventions, secondary prevention of stroke or ACS, and, possibly, prevention of priapism, pulmonary hypertension, and nonhealing ulcers. Transfusion is not indicated for uncomplicated pregnancy, routine painful episodes, minor surgery not requiring anesthesia, or asymptomatic anemia. Erythrocyte exchange transfusion is indicated for acute ischemic stroke, ACS with significant hypoxia, and multiorgan failure/hepatopathy as well as in persons in whom simple transfusion would increase the hemoglobin level to greater than 10 g/dL (100 g/L). Chronic transfusion can lead to iron overload, alloimmunization, and an increased risk for a delayed hemolytic transfusion reaction. Erythrocytes used in transfusion should be leukoreduced, hemoglobin S negative, and phenotypically matched for the E, C, and K antigens as well as for any known alloantibodies. Hemoglobin targets should remain less than 10 g/dL (100 g/L) to avoid hyperviscosity.
In a randomized trial, transfusion reduced the risk of pain crisis in pregnant women with SCD but showed no clear improvement in maternal mortality, perinatal mortality, or severe maternal morbidity (pulmonary embolism, chronic heart failure, ACS). Transfusion should be provided based on symptoms of anemia and not hemoglobin levels.
Erythropoiesis-stimulating agents (ESAs) are used to treat anemia in conditions in which bone marrow stimulation of erythrocyte production is inadequate, such as chronic kidney disease. However, in SCD, erythropoietin levels are typically high to augment bone marrow erythrocyte production in response to chronic hemolysis. Therefore, ESAs are not indicated for treatment of the anemia associated with SCD.