Chronic red blood cell exchange to prevent clinical complications in sickle cell disease

Transfusional iron overload in children with sickle cell anemia on chronic transfusion therapy for secondary stroke prevention

“…The finding of the lowest iron burden in children receiving erythrocytapheresis is not surprising, given that this method is associated with a reduction in net red cell loading [9,10]. Overall treatment costs also are lowered if chelation therapy can be avoided [20], further supporting the early use of erythrocytapheresis for long-term transfusion therapy in SCA when feasible.…”

“…A major concern with this therapy is the development of transfusional iron overload, which can cause endocrinopathies, liver inflammation and fibrosis, and cardiac toxicity [3][4][5], although some of these complications appear to occur less commonly in SCA compared with thalassemia patients [6,7]. The use of modified transfusion programs that allow higher pretransfusion hemoglobin S levels [8] and automated exchange transfusions may limit iron loading in SCA [9,10]. Chelation therapy also is an effective means of reducing iron burden, and the clinical availability of an oral chelator, deferasirox (DFX), since late 2005, may improve treatment adherence compared with the parenterally administered deferoxamine (DFO).…”

Spectra Optia® for Automated Red Blood Cell Exchange in Patients with Sickle Cell Disease: A NICE Medical Technology Guidance

“…Six studies compared the Spectra Optia system, or its predecessor the Cobe Spectra system, with manual RBC exchange [18–23]. These were all retrospective observational studies that did not use an experimental comparative design; three used historical controls [18, 21, 23], one was a before-and-after study [19] and two were between-centres studies [20, 22].…”

“…These were all retrospective observational studies that did not use an experimental comparative design; three used historical controls [18, 21, 23], one was a before-and-after study [19] and two were between-centres studies [20, 22]. Only two of the studies were peer reviewed [18, 20], one was a published letter [22], and three were reported as conference abstracts [19, 21, 23]. …”

Sickle cell crisis and pregnancy

“…This process typically involves the exchange of six units of donor packed red cells, and there is generally a rapid resolution of crisis symptomatology and ongoing sickling. 31 Most of the complications associated with transfusions are related to the risk of the blood products. Blood products given to these patients should be cytomegalovirus-negative, leukocytedepleted red cell units that are phenotypically matched for at least the C, D, E, and Kell blood groups.…”