Published Online
on April 20, 2006

A more recent version of this article appeared on June 1, 2006

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Submitted on January 13, 2006
Revised on March 7, 2006
Accepted on March 9, 2006

Regular Transfusion Lowers Plasma Free Hemoglobin in Children With Sickle-Cell Disease at Risk for Stroke

Nelson E. Lezcano PhD; Nadine Odo CCRC; Abdullah Kutlar MD; Donald Brambilla PhD; and Robert J. Adams MS, MD^*

From the Department of Neurology, Stroke Research Program, Department of Neurology School of Medicine, Medical College of Georgia, Augusta, GA (N.E.L., N.O., R.J.A.); the Department of Medicine and Sickle Cell Center (A.K.), Medical College of Georgia, Augusta, GA; and the New England Research Institutes (D.B.), Watertown, MA.

^* To whom correspondence should be addressed. E-mail: rjadams{at}mail.mcg.edu.

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 (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.

Key words: children • hemolysis • stroke

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