|
|
 Previous Article | Table of Contents | Next Article 
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.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
P. Monagle, E. Chalmers, A. Chan, G. deVeber, F. Kirkham, P. Massicotte, and A. D. Michelson
Antithrombotic Therapy in Neonates and Children: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition)
Chest,
June 1, 2008;
133(6_suppl):
887S - 968S.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
O. S. Platt
Hydroxyurea for the Treatment of Sickle Cell Anemia
N. Engl. J. Med.,
March 27, 2008;
358(13):
1362 - 1369.
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Mazumdar, M. M. Heeney, C. M. Sox, and T. A. Lieu
Preventing Stroke Among Children With Sickle Cell Anemia: An Analysis of Strategies That Involve Transcranial Doppler Testing and Chronic Transfusion
Pediatrics,
October 1, 2007;
120(4):
e1107 - e1116.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. Bernaudin, G. Socie, M. Kuentz, S. Chevret, M. Duval, Y. Bertrand, J.-P. Vannier, K. Yakouben, I. Thuret, P. Bordigoni, et al.
Long-term results of related myeloablative stem-cell transplantation to cure sickle cell disease
Blood,
October 1, 2007;
110(7):
2749 - 2756.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. A. Zimmerman, W. H. Schultz, S. Burgett, N. A. Mortier, and R. E. Ware
Hydroxyurea therapy lowers transcranial Doppler flow velocities in children with sickle cell anemia
Blood,
August 1, 2007;
110(3):
1043 - 1047.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. L. Sacco, R. Adams, G. Albers, M. J. Alberts, O. Benavente, K. Furie, L. B. Goldstein, P. Gorelick, J. Halperin, R. Harbaugh, et al.
Guidelines for Prevention of Stroke in Patients With Ischemic Stroke or Transient Ischemic Attack: A Statement for Healthcare Professionals From the American Heart Association/American Stroke Association Council on Stroke: Co-Sponsored by the Council on Cardiovascular Radiology and Intervention: The American Academy of Neurology affirms the value of this guideline.
Circulation,
March 14, 2006;
113(10):
e409 - e449.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. L. Sacco, R. Adams, G. Albers, M. J. Alberts, O. Benavente, K. Furie, L. B. Goldstein, P. Gorelick, J. Halperin, R. Harbaugh, et al.
Guidelines for Prevention of Stroke in Patients With Ischemic Stroke or Transient Ischemic Attack: A Statement for Healthcare Professionals From the American Heart Association/American Stroke Association Council on Stroke: Co-Sponsored by the Council on Cardiovascular Radiology and Intervention: The American Academy of Neurology affirms the value of this guideline.
Stroke,
February 1, 2006;
37(2):
577 - 617.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
V. G. Nolan, D. F. Wyszynski, L. A. Farrer, and M. H. Steinberg
Hemolysis-associated priapism in sickle cell disease
Blood,
November 1, 2005;
106(9):
3264 - 3267.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Gulbis, D. Haberman, D. Dufour, C. Christophe, C. Vermylen, F. Kagambega, F. Corazza, C. Devalck, M.-F. Dresse, K. Hunninck, et al.
Hydroxyurea for sickle cell disease in children and for prevention of cerebrovascular events: the Belgian experience
Blood,
April 1, 2005;
105(7):
2685 - 2690.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. A. Zimmerman, W. H. Schultz, J. S. Davis, C. V. Pickens, N. A. Mortier, T. A. Howard, and R. E. Ware
Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerated dose in children with sickle cell disease
Blood,
March 15, 2004;
103(6):
2039 - 2045.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. R. Buchanan, M. R. DeBaun, C. T. Quinn, and M. H. Steinberg
Sickle Cell Disease
Hematology,
January 1, 2004;
2004(1):
35 - 47.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. A. Lee and B. U. Mueller
Long-term Use of Hydroxyurea for Sickle Cell Anemia
JAMA,
August 13, 2003;
290(6):
753 - 754.
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. L. Weiner and C. Brugnara
Hydroxyurea and Sickle Cell Disease: A Chance for Every Patient
JAMA,
April 2, 2003;
289(13):
1692 - 1694.
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. F. Atweh, J. DeSimone, Y. Saunthararajah, H. Fathallah, R. S. Weinberg, R. L. Nagel, M. E. Fabry, and R. J. Adams
Hemoglobinopathies
Hematology,
January 1, 2003;
2003(1):
14 - 39.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. J. Helton, W. C. Wang, L. W. Wynn, R. B. Khan, and R. G. Steen
The Effect of Hydroxyurea on Vasculopathy in a Child with Sickle Cell Disease
AJNR Am. J. Neuroradiol.,
November 1, 2002;
23(10):
1692 - 1696.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. R. Dobson, K. R. Holden, P. J. Nietert, J. K. Cure, J. H. Laver, D. Disco, and M. R. Abboud
Moyamoya syndrome in childhood sickle cell disease: a predictive factor for recurrent cerebrovascular events
Blood,
May 1, 2002;
99(9):
3144 - 3150.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. H. Pegelow, E. A. Macklin, F. G. Moser, W. C. Wang, J. A. Bello, S. T. Miller, E. P. Vichinsky, M. R. DeBaun, L. Guarini, R. A. Zimmerman, et al.
Longitudinal changes in brain magnetic resonance imaging findings in children with sickle cell disease
Blood,
April 15, 2002;
99(8):
3014 - 3018.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Ferster, P. Tahriri, C. Vermylen, G. Sturbois, F. Corazza, P. Fondu, C. Devalck, M. F. Dresse, W. Feremans, K. Hunninck, et al.
Five years of experience with hydroxyurea in children and young adults with sickle cell disease
Blood,
June 1, 2001;
97(11):
3628 - 3632.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. J. Adams
Stroke Prevention and Treatment in Sickle Cell Disease
Arch Neurol,
April 1, 2001;
58(4):
565 - 568.
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. J. Adams, K. Ohene-Frempong, and W. Wang
Sickle Cell and the Brain
Hematology,
January 1, 2001;
2001(1):
31 - 46.
[Abstract]
[Full Text]
[PDF]
|
|
|
| |
