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Blood, Vol. 94 No. 9 (November 1), 1999:
pp. 3022-3026
Hydroxyurea as an Alternative to Blood Transfusions for the
Prevention of Recurrent Stroke in Children With Sickle Cell Disease
Russell E. Ware,
Sherri A. Zimmerman, and
William H. Schultz
From the Duke Pediatric Sickle Cell Program and the Division of
Hematology-Oncology, Department of Pediatrics, Duke University Medical
Center, Durham, NC.
Children with sickle cell disease (SCD) and stroke receive chronic
transfusions to prevent stroke recurrence. Transfusion risks including
infection, erythrocyte allosensitization, and iron overload suggest a
need for alternative therapies. We previously used hydroxyurea (HU) and
phlebotomy in two young adults with SCD and stroke as an alternative to
transfusions. We have now prospectively discontinued
transfusions in 16 pediatric patients with SCD and stroke. Reasons to
discontinue transfusions included erythrocyte alloantibodies or
autoantibodies, recurrent stroke on transfusions, iron overload,
noncompliance, and deferoxamine allergy. HU was started at 15 mg/kg/d
and escalated to 30 mg/kg/d based on hematologic toxicity. Patients
with iron overload underwent phlebotomy. The children have been off
transfusions 22 months, (range, 3 to 52 months). Their average HU dose
is 24.9 ± 4.2 mg/kg/d, hemoglobin concentration is 9.4 ± 1.3 g/dL, and mean corpuscular volume (MCV) is 112 ± 9 fL. Maximum percentage fetal hemoglobin (%HbF) is
20.6% ± 8.0% and percentage HbF-containing erythrocytes (%F cells) is 79.3% ± 14.7%. Fourteen patients
underwent phlebotomy with an average of 8,993 mL (267 mL/kg)
removed. Serum ferritin has decreased from 2,630 to 424 ng/mL, and 4 children have normal ferritin values. Three patients (19%) had
neurological events considered recurrent stroke, each 3 to 4 months
after discontinuing transfusions, but before maximal HU effects. These
preliminary data suggest some children with SCD and stroke may
discontinue chronic transfusions and use HU therapy to prevent stroke
recurrence. Phlebotomy is well-tolerated and significantly reduces iron
overload. Modifications in HU therapy to raise HbF more rapidly might
increase protection against stroke recurrence.
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