Transfusion Management in Patients with Hemoglobinopathies
This chapter discusses the management of hemoglobinopathy patients with transfusion. The patients with hemoglobinopathies require special considerations due to several reasons: they require acute and chronic, simple and exchange transfusions, need phenotypically matched transfusions, they may develop allo and autoantibodies that can be difficult to identify, they require specialized procedures such as prophylactic or urgent RBC exchange. The chapter focuses on the most common hereditary chronic anemia/hemoglobinopathy patients that require lifelong RBC transfusion support, namely sickle cell disease (SCD) or thalassemia. SCD is mentioned to be the most prevalent genetic disorder in the African American population and affects approximately 80,000 people in the US. A key component of the treatment of SCD patients is RBC transfusion. The goals of RBC transfusion in SCD patients are to improve oxygen-carrying capacity by increasing Hgb AA concentration, decreasing blood viscosity and improving blood flow by decreasing or diluting the Hgb S containing RBCs and suppressing endogenous erythropoiesis. © 2009 Elsevier Inc. All rights reserved.