Abstract
This chapter outlines the role of and methods for transfusion in children with sickle cell disease (SCD) and thalassemia. The transfusion goals are reviewed along with a thorough listing of appropriate indications for transfusion in children with SCD. Methods of transfusion therapy, including acute simple transfusion, chronic simple transfusion, and both manual and automated red blood cell (RBC) exchange therapy are explained, and formulas are provided for calculating the appropriate transfusion and exchange of RBC volumes. For both SCD and thalassemia patients, adverse effects of transfusion and strategies for their prevention and management are also discussed in the chapter. The goals of RBC transfusion in SCD patients are to improve oxygen-carrying capacity by increasing the total hemoglobin concentration; to decrease blood viscosity and improve blood flow by diluting RBCs that contain sickle hemoglobin; and to suppress endogenous erythropoiesis by increasing tissue oxygenation. The goals of transfusion therapy in children with ß-thalassemia major are to increase oxygen-carrying capacity by correcting the anemia, prevent progressive hypersplenism, suppress erythropoiesis, and reduce increased gastrointestinal absorption of iron.
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