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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.
Submitted June 23, 2003
Accepted November 11, 2003
Magnetic resonance angiography in children with sickle cell disease and abnormal transcranial doppler ultrasonography enrolled in the "STOP" study
Miguel R Abboud*, Joel Cure, Suzanne Granger, Diane Gallagher, Lewis Hsu, Winfred Wang, Gerald Woods, Brian Berman, Don Brambilla, Charles Pegelow, Jonathan Lewin, Robert A Zimmerman, and Robert J Adams
Department of Pediatrics, Medical University of South Carolina, Charleston, SC, USA
Department of Radiology, Medical University of South Carolina, Charleston, SC, USA
The New England Research Institute, Watertown, MA, USA
Sickle Cell Center, Emory Univerity School of Medicine, Atlanta, GA, USA
Department of Hematology/Oncology, St Jude Children's Research Hospital, Memphis, TN, USA
Deparment of Hematology/Oncology, Children's Mercy Hospital of Kansas City, Kansas City, MO, USA
Department of Pediatric Hematology and Oncology, Rainbow Babies and Children's Hospital, Cleveland, OH, USA
Department of Pediatrics, University of Miami, Miami, FL, USA
Department of Radiology, University Hospitals of Cleveland, Cleveland, OH, USA
Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
Department of Neurology, Medical College of Georgia, Augusta, GA, USA
* Corresponding author; email: abboudm{at}aub.edu.lb.
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)  200 cm/s measured by transcranial Doppler (TCD). In STOP 232 brain magnetic resonance angiograms (MRA) were performed on 100 patients, 47 in the transfusion arm and 53 in the standard care arm. Baseline MRAs were interpreted as normal in 75, mild stenosis in 4 and severe stenosis in 21 patients. Among 35 patients who had MRA within 30 days of randomization, the TAMV was significantly higher in 7 with severe stenosis compared to 28 with normal MRA or mid stenosis (276.7±34 vs. 215±15.6 cm/s, p<0.001). In the standard care arm 4 of 13 patients with abnormal MRA developed strokes as opposed to 5 of 40 patients with normal MRA (p= 0.03). In this arm TAMV became normal (<170 cm/sec) or conditional (170-199 cm/sec) in 26 of 38 patients with normal or mildly abnormal baseline MRA but remained abnormal in 8 of 10 patients with severely abnormal baseline MRAs. These results suggest that TCD detects flow abnormalities, that predict stroke risk, before MRA lesions become evident. Furthermore, patients with abnormal MRA and higher TCD velocities are at higher risk for stroke and their cerebral TAMVs are unlikely to decrease without transfusion.
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