Abstract
Red blood cell exchanges are frequently used to treat and prevent cerebrovascular complications in patients with sickle cell anemia (SCA). However, the low weight of young children represents serious concerns for this procedure. The Spectra Optia device can perform automatic priming using red blood cells (RBCs) (RCE/RBC-primed) which could allow RBC exchanges (RCE) to be performed in young children without hypovolemic complications, but this method requires evaluation. We prospectively analyzed the clinical safety of the RCE/RBC-primed procedure in 12 SCA low-weight children under either a chronic RCE program or emergency treatment over 65 sessions. We monitored grade 2 adverse events (AEs) such as a decrease in blood pressure, increase in heart rate, fainting sensation, or transfusion reactions and identified the critical times during the sessions in which AEs could occur. Post-apheresis hematocrit (Hct) and a fraction of cell remaining (FCR) values were compared to the expected values. We also compared the impact of automatic RCE (n = 7) vs. RCE/RBC-primed (n = 8) on blood viscosity and RBC rheology. A low incidence of complications was observed in the 65 RCE sessions with only seven episodes of transient grade 2 AEs. Post-apheresis Hct and FCR reached expected values with the RCE/RBC-primed method. Both the automatic and priming procedures improved RBC deformability and decreased the sickling tendency during deoxygenation. Blood rheological features improved in both RCE/RBC-primed and automatic RCE without priming conditions. The RCE/RBC-primed procedure provides blood rheological benefits, and is safe and efficient to treat, notably in young children with SCA in prophylactic programs or curatively when a SCA complication occurs.
Highlights
Sickle cell anemia is caused by a point mutation in the seventh position of the β-globin gene leading to the production of abnormal hemoglobin called HbS
In most children (10/12), extracorporeal volume (EV) of the circuit represented a high volume related to their total blood volume (TBV) (EV/TBV ratio greater than 15%) while lower EV/TBV was associated with low native Hct (nHct) (21 and 22% for patients 6 and 12, respectively) were found in two of them
The principle of RBC exchange (RCE) is to prevent the occurrence of sickle cell anemia (SCA) complications by replacing abnormal red blood cells (RBC) with normal RBCs so that the HbS level does not exceed a defined threshold between two sessions [15, 23]
Summary
This replacement can be achieved by transfusions associated with manual bloodletting. This methodology allows a limited exchange of abnormal RBCs and may generate blood hyperviscosity, which in turn may result in vascular complications, and does not prevent iron overload [6]. RBC exchange (RCE) using an apheresis device can replace a greater number of RBCs, is more efficient in preventing the occurrence of SCA complications when regularly performed, decreases whole blood viscosity, and limits the risk of iron overload [6,7,8,9,10]
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