Assessment of Implementation of the Automated Erythrocytapheresis in the Outcome of Egyptian Sickle Cell Disease Patients: Single Center Experience

There is a wide scoop for treatment of SCD ranging from medical treatment with hydroxyurea, simple transfusion with chelation therapy, exchange transfusion either manual or automated and stem cell transplantation which need highly equipped institutes.

The goals of treatment of SCD are symptom control with early detection and management of disease complications. Although several pharmacological agents have been studied for the treatment of SCD, the only drug currently approved by the US Food and Drug Administration (FDA) for the treatment of SCD is hydroxyurea. Allogeneic bone marrow transplantation (BMT) can cure SCD, but it is difficult to decide which patients should be offered BMT. Many risks are associated with BMT, and the risk-to-benefit ratio must be assessed carefully. The lack of availability of a matched donor may limit the utility of BMT. Transfusions are not needed for the usual anemia or episodes of pain associated with SCD. Urgent replacement of blood is often required for sudden severe anemia and regular blood transfusions are used for primary and secondary stroke prevention in children with SCD. With continued transfusion, iron overload inevitably develops and can result in heart and liver failure, and multiple other complications necessitate the use of chelation therapy.

Erythrocytapheresis is an automated red cell exchange procedure that removes blood that contains HbS from the patient while simultaneously replacing that same volume with packed red cells free of HbS. Transfusion usually consists of sickle-negative, leuco-reduced, and phenotypically matched blood for red cell antigens. Erythrocytapheresis thus has the advantage of controlling iron accumulation in patients with SCD who undergo long-term transfusion, as well as the ability to allow rapid reduction of HbS concentrations to less than 30% without significantly increasing total hemoglobin concentration post transfusion. This precision is achieved because, before the start of the transfusion, the computer in the pheresis machine calculates the expected amount of packed RBCs required to obtain a specific post-transfusion hemoglobin level, using various physiologic parameters (eg, height, weight, Hb level). Further, erythrocytapheresis requires less time than simple transfusion of similar blood volumes. Although erythrocytapheresis is more expensive than simple transfusion, the additional costs associated with simple transfusions (ie, those of chelation and organ damage due to iron overload) make erythrocytapheresis more cost-effective than simple transfusion programs. Central venous access devices can safely be used for long-term erythrocytapheresis in patients with SCD with a low rate of complications.