ABSTRACT

Blood transfusion su p p o rt in patients w ith hered itary anem ias differs in th a t (1) m any o f these patien ts receive their first transfusion in early life, and since long te rm chronic red cell transfusion is often em ployed iron accum ulation by adolescence presents a clinical p roblem (2) although the need for transfusion is partly related to the need to increase oxygen delivery, an im p o rtan t aspect is the suppression o f erythropoiesis by elevating the hem atocrit, w hich has the effect o f preventing developm ental skeletal m alform ations and (3) use o f b lood products o th er th an red b lood cells is unusual.