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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Red blood cell exchange in children with sickle cell disease
Narcisse Elenga1,2, Vincent Vantilcke3, Elise Martin4
1Sickle Cell Disease Center, Centre Hospitalier de Cayenne, Rue des Flamboyants, BP 6006, 97306, Cayenne Cedex, French Guiana. elengafr@yahoo.fr.
Insights
Automated red blood cell exchange (RBCx) effectively treats sickle cell disease (SCD) complications in children. This apheresis method safely reduces HbS levels below 30%, improving patient outcomes and preventing serious health issues.
Area of Science:
- Hematology
- Pediatric Medicine
- Apheresis Technology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder causing severe complications in children.
- Red blood cell exchange (RBCx) is a treatment option, but its efficacy in pediatric SCD requires further assessment.
- Automated apheresis systems offer potential for efficient RBCx delivery.
Purpose of the Study:
- To evaluate the effectiveness and safety of automated red blood cell exchange (RBCx) using the Spectra Optia® system in pediatric patients with sickle cell disease (SCD).
- To determine optimal parameters for RBCx in managing both acute and chronic complications of SCD in children.
- To establish target post-exchange hemoglobin S (HbS) levels for effective treatment.
Main Methods:
- Conducted a retrospective analysis of 649 RBCx sessions in 75 children with SCD (median age 10 years) treated with the Spectra Optia® system.
- Utilized femoral double-lumen central venous catheters when peripheral venous access was limited, recommending heparin locking.
- Analyzed efficacy based on achieving post-exchange HbS levels below 30% for both acute and chronic management strategies.
Main Results:
- Automated RBCx was successfully employed in 75 children with SCD, managing both acute and chronic complications.
- Achieving a post-exchange HbS level <30% was a key indicator of treatment success.
- For chronic management, specific parameters included Hb levels of 10-11 g/dL, exchange volume ≥32 mL/kg, and intervals ≤30 days.
- For acute management, optimal results were achieved with Hb levels ≥10 g/dL and total exchange volume ≥35 mL/kg (AUC 0.84).
Conclusions:
- Automated red blood cell exchange (RBCx) is a safe and effective therapeutic option for managing acute and chronic complications in children with sickle cell disease (SCD).
- The Spectra Optia® system facilitates efficient RBCx, with specific transfusion parameters ensuring optimal reduction of HbS levels.
- Careful management of venous access and adherence to target HbS levels are crucial for successful RBCx therapy in pediatric SCD patients.
Abstract:
The aim of our study was to assess the efficacy of red blood cell exchange (RBCx) using a Spectra Optia® automated apheresis system in children with sickle cell disease (SCD). We used automated RBCx to treat acute and chronic complications in 75 children with SCD who had a median age of 10 years [7-13]. We analyzed 649 RBCx sessions. Peripheral venous access was limited in a number of the children, and thus a femoral double-lumen central venous catheter was required. We recommend heparin locking with 500 units in each lumen of the catheter. To prevent complications, we ensured that all patients had achieved a post-RCE HbS level of < 30%. For chronic transfusion, with a post-RCE Hb level of approximately 10-11 g/dL, a blood exchange volume of ≥ 32 mL/kg, and an interval between each RBCx procedure of ≤ 30 days, the residual HbS level was maintained below 30%. For acute transfusion, a post-exchange Hb level ≥ 10 g/dL (p < 0.001) and a total exchange volume ≥ 35 mL/kg (p = 0.001) were the best way to reduce HbS to < 30%. AUC was 0.84. Our results show that erythrocytapheresis was useful and safe for children with SCD.
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