Prevention of a First Stroke by Transfusions in Children with Sickle Cell Anemia and Abnormal Results on Transcranial Doppler Ultrasonography
Robert J. Adams, M.D., Virgil C. McKie, M.D., Lewis Hsu, M.D., Ph.D., Beatrice Files, M.D., Elliott Vichinsky, M.D., Charles Pegelow, M.D., Miguel Abboud, M.D., Dianne Gallagher, M.S., Abdullah Kutlar, M.D., Fenwick T. Nichols, M.D., Duane R. Bonds, M.D., Donald Brambilla, Ph.D., Gerald Woods, M.D., Nancy Olivieri, M.D., Catherine Driscoll, M.D., Scott Miller, Winfred Wang, M.D., Anne Hurlett, M.D., Charles Scher, M.D., Brian Berman, M.D., Elizabeth Carl, B.A., Anne M. Jones, R.N., E. Steve Roach, M.D., Elizabeth Wright, Ph.D., Robert A. Zimmerman, M.D., and Myron Waclawiw, Ph.D.
Background Blood transfusions prevent recurrent stroke in childrenwith sickle cell anemia, but the value of transfusions in preventinga first stroke is unknown. We used transcranial Doppler ultrasonographyto identify children with sickle cell anemia who were at highrisk for stroke and then randomly assigned them to receive standardcare or transfusions to prevent a first stroke.
Methods To enter the study, children with sickle cell anemiaand no history of stroke had to have undergone two transcranialDoppler studies that showed that the time-averaged mean blood-flowvelocity in the internal carotid or middle cerebral artery was200 cm per second or higher. The patients were randomly assignedto receive standard care or transfusions to reduce the hemoglobinS concentration to less than 30 percent of the total hemoglobinconcentration. The incidence of stroke (cerebral infarctionor intracranial hemorrhage) was compared between the two groups.
Results A total of 130 children (mean [±SD] age, 8.3±3.3years) were enrolled; 63 were randomly assigned to receive transfusions,and 67 to receive standard care. At base line, the transfusiongroup had a slightly lower mean hemoglobin concentration (7.2vs. 7.6 g per deciliter, P=0.001) and hematocrit (20.4 vs. 21.7percent, P=0.002). Ten patients dropped out of the transfusiongroup, and two patients crossed over from the standard-caregroup to the transfusion group. There were 10 cerebral infarctionsand 1 intracerebral hematoma in the standard-care group, ascompared with 1 infarction in the transfusion group a 92 percent difference in the risk of stroke (P<0.001).This result led to the early termination of the trial.
Conclusions Transfusion greatly reduces the risk of a firststroke in children with sickle cell anemia who have abnormalresults on transcranial Doppler ultrasonography.
Source Information
From the Departments of Neurology (R.J.A., F.T.N.), Pediatric Hematology and Oncology (V.C.M.), and Medicine (A.K.), Medical College of Georgia, Augusta; the Sickle Cell Center, Emory University School of Medicine, Atlanta (L.H.); the Department of Pediatric Hematology and Oncology, East Carolina University School of Medicine, Greenville, N.C. (B.F.); the Department of Hematology and Oncology, Children's Hospital Oakland, Oakland, Calif. (E.V.); the Sickle Cell Center, University of Miami School of Medicine, Miami (C.P.); the Pediatric Sickle Cell Program, Medical University of South Carolina, Charleston (M.A.); the New England Research Institutes, Watertown, Mass. (D.G., D.B.); and the National Heart, Lung, and Blood Institute, Bethesda, Md. (D.R.B.). Other authors were Gerald Woods, M.D. (Department of Hematology and Oncology, Children's Mercy Hospital, Kansas City, Mo.), Nancy Olivieri, M.D. (Department of Hematology and Oncology, Hospital for Sick Children, Toronto), Catherine Driscoll, M.D. (Department of Hematology and Oncology, Children's National Medical Center, Washington, D.C.), Scott Miller (Pediatric Sickle Cell Program, State University of New York Health Science Center at Brooklyn, Brooklyn), Winfred Wang, M.D. (Department of Hematology and Oncology, St. Jude Children's Research Hospital, Memphis, Tenn.), Anne Hurlett, M.D. (Department of Pediatric Hematology, ColumbiaPresbyterian Medical Center, New York), Charles Scher, M.D. (Department of Pediatric Hematology and Oncology, Tulane University Medical School, New Orleans), Brian Berman, M.D. (Department of Pediatric Hematology and Oncology, Rainbow Babies and Children's Hospital, Cleveland), Elizabeth Carl, B.A., and Anne M. Jones, R.N. (Department of Neurology, Medical College of Georgia, Augusta), E. Steve Roach, M.D. (Department of Pediatric Neurology, University of Texas Southwestern Medical Center, Dallas), Elizabeth Wright, Ph.D. (New England Research Institutes, Watertown, Mass.), Robert A. Zimmerman, M.D. (Department of Neuroradiology, Children's Hospital of Philadelphia, Philadelphia), and Myron Waclawiw, Ph.D. (National Heart, Lung, and Blood Institute, Bethesda, Md.).
Address reprint requests to Dr. Adams at the Department of Neurology, Medical College of Georgia, 1467 Harper St., HB-2060, Augusta, GA 30912-3200.
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Walters, M. C., Nienhuis, A. W., Vichinsky, E.
(2002). Novel Therapeutic Approaches in Sickle Cell Disease. ASH Education Book
2002: 10-34
[Abstract][Full Text]
Eldor, A., Rachmilewitz, E. A.
(2002). The hypercoagulable state in thalassemia. Blood
99: 36-43
[Abstract][Full Text]
Pegelow, C. H., Wang, W., Granger, S., Hsu, L. L., Vichinsky, E., Moser, F. G., Bello, J., Zimmerman, R. A., Adams, R. J., Brambilla, D., for the STOP Trial,
(2001). Silent Infarcts in Children With Sickle Cell Anemia and Abnormal Cerebral Artery Velocity. Arch Neurol
58: 2017-2021
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Moser, C., Nussbaum, E., Cooper, D. M.
(2001). Plastic Bronchitis and the Role of Bronchoscopy in the Acute Chest Syndrome of Sickle Cell Disease. Chest
120: 608-613
[Abstract][Full Text]
Lonergan, G. J., Cline, D. B., Abbondanzo, S. L.
(2001). Sickle Cell Anemia. RadioGraphics
21: 971-994
[Abstract][Full Text]
Cheung, A. T. W., Harmatz, P., Wun, T., Chen, P. C. Y., Larkin, E. C., Adams, R. J., Vichinsky, E. P.
(2001). Correlation of abnormal intracranial vessel velocity, measured by transcranial Doppler ultrasonography, with abnormal conjunctival vessel velocity, measured by computer-assisted intravital microscopy, in sickle cell disease. Blood
97: 3401-3404
[Abstract][Full Text]
Malouf, A. J. Jr, Hamrick-Turner, J. E., Doherty, M. C., Dhillon, G. S., Iyer, R. V., Smith, M. G.
(2001). Implementation of the STOP Protocol for Stroke Prevention in Sickle Cell Anemia by Using Duplex Power Doppler Imaging. Radiology
219: 359-365
[Abstract][Full Text]
Adams, R. J.
(2001). Stroke Prevention and Treatment in Sickle Cell Disease. Arch Neurol
58: 565-568
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Schmugge, M., Frischknecht, H., Yonekawa, Y., Baumgartner, R. W., Boltshauser, E., Humbert, J.
(2001). Stroke in hemoglobin (SD) sickle cell disease with moyamoya: successful hydroxyurea treatment after cerebrovascular bypass surgery. Blood
97: 2165-2167
[Abstract][Full Text]
Goldstein, L. B., Adams, R., Becker, K., Furberg, C. D., Gorelick, P. B., Hademenos, G., Hill, M., Howard, G., Howard, V. J., Jacobs, B., Levine, S. R., Mosca, L., Sacco, R. L., Sherman, D. G., Wolf, P. A., del Zoppo, G. J., Members,
(2001). Primary Prevention of Ischemic Stroke : A Statement for Healthcare Professionals From the Stroke Council of the American Heart Association. Circulation
103: 163-182
[Full Text]
Goldstein, L. B., Adams, R., Becker, K., Furberg, C. D., Gorelick, P. B., Hademenos, G., Hill, M., Howard, G., Howard, V. J., Jacobs, B., Levine, S. R., Mosca, L., Sacco, R. L., Sherman, D. G., Wolf, P. A., del Zoppo, G. J.
(2001). Primary Prevention of Ischemic Stroke : A Statement for Healthcare Professionals From the Stroke Council of the American Heart Association. Stroke
32: 280-299
[Full Text]
Adams, R. J., Ohene-Frempong, K., Wang, W.
(2001). Sickle Cell and the Brain. ASH Education Book
2001: 31-46
[Abstract][Full Text]
Wayne, A. S., Schoenike, S. E., Pegelow, C. H.
(2000). Financial analysis of chronic transfusion for stroke prevention in sickle cell disease. Blood
96: 2369-2372
[Abstract][Full Text]
Hassan, A., Markus, H. S.
(2000). Genetics and ischaemic stroke. Brain
123: 1784-1812
[Abstract][Full Text]
Harmatz, P., Butensky, E., Quirolo, K., Williams, R., Ferrell, L., Moyer, T., Golden, D., Neumayr, L., Vichinsky, E.
(2000). Severity of iron overload in patients with sickle cell disease receiving chronic red blood cell transfusion therapy. Blood
96: 76-79
[Abstract][Full Text]
Styles, L. A., Hoppe, C., Klitz, W., Vichinsky, E., Lubin, B., Trachtenberg, E.
(2000). Evidence for HLA-related susceptibility for stroke in children with sickle cell disease. Blood
95: 3562-3567
[Abstract][Full Text]
Nietert, P. J., Abboud, M. R., Silverstein, M. D., Jackson, S. M.
(2000). Bone marrow transplantation versus periodic prophylactic blood transfusion in sickle cell patients at high risk of ischemic stroke: a decision analysis. Blood
95: 3057-3064
[Abstract][Full Text]