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Blood, 1 April 2004, Vol. 103, No. 7, pp. 2822-2826.
Prepublished online as a Blood First Edition Paper on December 18, 2003; DOI 10.1182/blood-2003-06-1972.
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RED CELLS
Magnetic resonance angiography in children with sickle cell disease and abnormal transcranial Doppler ultrasonography findings enrolled in the STOP study
Miguel R. Abboud,
Joel Cure,
Suzanne Granger,
Dianne Gallagher,
Lewis Hsu,
Winfred Wang,
Gerald Woods,
Brian Berman,
Don Brambilla,
Charles Pegelow,
Jonathan Lewin,
Robert A. Zimmermann, and
Robert J. Adams
From the Departments of Pediatrics and Radiology, Medical University of South Carolina, Charleston; New England Research Institute, Watertown, MA; Children's Mercy Hospital of Kansas City, MO; Sickle Cell Center, Emory University School of Medicine, Atlanta, GA; Department of Hematology/Oncology, St Jude Children's Research Hospital, Memphis, TN; Department of Pediatric Hematology and Oncology, Rainbow Babies and Children's Hospital, Cleveland, OH; University of Miami School of Medicine, FL; University Hospitals of Cleveland, OH; Department of Radiology, Children's Hospital of Philadelphia, PA; and Department of Neurology, Medical College of Georgia, Augusta.
The stroke prevention study in sickle cell disease (STOP) demonstrated a 90% reduction in stroke risk with transfusion among patients with time-averaged mean cerebral blood velocity (TAMV) of 200 cm/s or more as measured by transcranial Doppler (TCD). In STOP, 232 brain magnetic resonance angiograms (MRAs) were performed on 100 patients, 47 in the transfusion arm and 53 in the standard care arm. Baseline MRA findings were interpreted as normal in 75 patients and as indicating mild stenosis in 4 patients and severe stenosis in 21 patients. Among 35 patients who underwent magnetic resonance angiography within 30 days of random assignment, the TAMV was significantly higher in 7 patients with severe stenosis compared with 28 patients with normal MRA findings or mild stenosis (276.7 ± 34 vs 215 ± 15.6 cm/s;P < .001). In the standard care arm, 4 of 13 patients with abnormal MRA findings had strokes compared with 5 of 40 patients with normal MRA findings (P = .03). In this arm, TAMV became normal (less than 170 cm/s) or conditional (170-199 cm/s) in 26 of 38 patients with normal or mildly abnormal baseline MRA but remained abnormal in 8 of 10 patients with severely abnormal baseline MRA. These results suggest that TCD often detects flow abnormalities indicative of stroke risk before MRA lesions become evident. Furthermore, patients with abnormal MRA findings and higher TCD velocities are at higher risk for stroke, and their cerebral TAMVs are unlikely to decrease without transfusion. (Blood. 2004;103:2822-2826)
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