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Updated: Aug 9, 2026
Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Regular transfusion lowers plasma free hemoglobin in children with sickle-cell disease at risk for stroke
Nelson E Lezcano1, Nadine Odo, Abdullah Kutlar
1Department of Neurology, School of Medicine, Medical College of Georgia, Augusta, GA 30912, USA.
Background And Purpose:
Intravascular hemolysis releases large amounts of free hemoglobin (PFH) in plasma of sickle-cell disease (SCD) patients. PFH has been associated with harmful endothelial actions including scavenging nitric oxide (NO). Whether PFH plays a role in stroke in SCD has not been examined.
Methods:
Serum levels of PFH, lactate dehydrogenase, and total bilirubin were measured in stored sera from children at risk for stroke treated in a randomized controlled trial of regular red cell transfusion (STOP study). Baseline and post-treatment (approximately 1 year of transfusion) were compared to determine whether treatment (which reduces stroke risk by 90%) was associated with reduction in markers of hemolysis.
Results:
Baseline serum PFH values did not differ between treatment groups. PFH declined with repeated transfusion from 78.7+/-8.2 mg/dL to 34.4+/-3.4 mg/dL (P<0.001). With only episodic or no transfusion the drop was smaller: 80.9+/-7.5 to 62.8+/-5.0 (P=0.019). The decrease was larger in those with regular transfusion (56% versus 22%; P<0.001). Reduction of lactate dehydrogenase and total bilirubin was observed only in those on regular transfusion.
Conclusions:
Regular transfusion which lowers stroke risk is associated with a significant reduction in PFH. A role for PFH in promoting stroke in SCD should be investigated.
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