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Case Report
PEDIATRICS

The Effect of Hydroxyurea on Vasculopathy in a Child with Sickle Cell Disease

Kathleen J. Heltona,e, Winfred C. Wangb,f, Lynn W. Wynnb,f, Raja B. Khanc,d and R. Grant Steena,e,f,g

a Department of Diagnostic Imaging, St Jude Children’s Research Hospital, Memphis
b Department of Hematology, St Jude Children’s Research Hospital, Memphis
c Department of Neurology, St Jude Children’s Research Hospital, Memphis
d Department of Neuro-Oncology, St Jude Children’s Research Hospital, Memphis
e Department of Radiology, University of Tennessee School of Medicine, Memphis
f Department of Pediatrics, University of Tennessee School of Medicine, Memphis
g Department of Biomedical Engineering, University of Tennessee School of Medicine, Memphis

Address reprint requests to R. Grant Steen, Department of Diagnostic Imaging, St Jude, Children’s Research Hospital, 332 N. Lauderdale, Memphis, TN 38105-2794

Summary: We report serial CNS findings in a girl with sickle cell disease and stroke. Religious considerations precluded transfusion and bone marrow transplantation; therefore, she received single-agent hydroxyurea therapy for almost 6 years. MR angiography showed that vascular patency improved, although diffuse cerebral atrophy slowly worsened. Hydroxyurea can be effective in treating vasculopathy, but it might not prevent the progression of parenchymal damage in advanced disease.