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Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease

  1. Winfred C Wang1,*,
  2. Kerry Dwan2

Editorial Group: Cochrane Cystic Fibrosis and Genetic Disorders Group

Published Online: 14 NOV 2013

Assessed as up-to-date: 1 NOV 2013

DOI: 10.1002/14651858.CD003146.pub2


How to Cite

Wang WC, Dwan K. Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease. Cochrane Database of Systematic Reviews 2013, Issue 11. Art. No.: CD003146. DOI: 10.1002/14651858.CD003146.pub2.

Author Information

  1. 1

    St Jude Children's Research Hospital, Department of Hematology, Memphis, Tennessee 38105, USA

  2. 2

    University of Liverpool, Department of Biostatistics, Liverpool, England, UK

*Winfred C Wang, Department of Hematology, St Jude Children's Research Hospital, 262 Danny Thomas Place, Mail Stop 800, Memphis, Tennessee 38105, USA. Winfred.Wang@stjude.org.

Publication History

  1. Publication Status: New search for studies and content updated (no change to conclusions)
  2. Published Online: 14 NOV 2013

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References

References to studies included in this review

  1. References to studies included in this review
  2. References to ongoing studies
  3. Additional references
  4. References to other published versions of this review
STOP 1998 {published data only}
  • Abboud M, Cure J, Gallagher D, Berman B, Hsu L, Wang W, et al. Magnetic resonance angiography (MRA) in children with abnormal transcranial doppler (TCD) velocities in the STOP study [abstract]. Blood 1999;94(10 Suppl 1):645a.
  • Abboud MR, Cure J, Gallagher D, Berman B, Hsu L, Wang W, et al. Magnetic resonance angiography in children with abnormal TCD in the STOP study [abstract]. Proceedings of the 23rd Annual Meeting of the National Sickle Cell Disease Program. 1999:49.
  • Abboud MR, Cure J, Granger S, Gallagher D, Hsu L, Wang W, et al. Magnetic resonance angiography in children with sickle cell disease and abnormal transcranial Doppler ultrasonography findings enrolled in the STOP study. Blood 2004;103(7):2822-6.
  • Abound MR, Yim E, Musallam KM, Adams RJ. Discontinuing prophylactic transfusions increases the risk of silent brain infarction in children with sickle cell disease: data from STOP II. Blood 2011;118(4):894-8.
  • Adamkiewicz T, Abboud M, Barredo J, Cavalier ME, Peterson J, Rackoff B, et al. Serum Ferritin in children with SCD on chronic transfusion: correlation with serum alanine aminotransferase and liver iron (STOP/STOP II liver iron ancillary study) [abstract]. Proceedings of the 35th Annivesary Convention of the National Sickle Celll Disease Program and the Siclkle Cell Disease Association of America; 2007 Sep 17-22; Washington DC, USA. 2007:316.
  • Adamkiewicz T, Abboud MR, Barredo JC, Kirby-Allen M, Alvarez OA, Casella JF, et al. Serum ferritin in children with sickle cell disease on chronic transfusion: measure of iron overload or end organ injury? STOP/ STOP II liver iron ancillary study [abstract]. Blood 2006;108(11):Abstract no: 791.
  • Adamkiewicz TV, Abboud MR, Paley C, Olivieri N, Kirby-Allen M, Vichinsky E, et al. Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury. Blood 2009;114(21):4632-8.
  • Adams RJ. Lessons from the stroke prevention trial in sickle cell anemia (STOP) study. Journal of Child Neurology 2000;15(5):344-9.
  • Adams RJ, Brambilla DJ. Stroke prevention trial in sickle cell anemia: STOP [abstract]. Proceedings of the 21st Annual Meeting of the National Sickle Cell Disease Program. 1996:19.
  • Adams RJ, Brambilla DJ, Granger S, Gallagher D, Vichinsky E, Abboud MR, et al. Stroke and conversion to high risk in children screened with transcranial Doppler ultrasound during the STOP study. Blood 2004;103(10):3689-94.
  • Adams RJ, Brambilla DJ, McKie V, Files B, Vichinsky E, Abboud M, et al. Risk of stroke in children with sickle cell disease and abnormal transcranial doppler ultrasound (TCD) [abstract]. Blood 1999;94(10 Suppl 1):419a.
  • Adams RJ, Brambilla DJ, McKie V, Files B, Vichinsky E, Abboud M, et al. Stroke prevention in sickle cell disease (STOP): Baseline characteristics of trial patients [abstract]. Proceedings of the 22nd Annual Meeting of the National Sickle Cell Disease Program. 1997:41.
  • Adams RJ, Brambilla DJ, McKie V, Files B, Vichinsky E, Abboud M, et al. Stroke prevention in sickle cell disease (STOP): Final results [abstract]. Proceedings of the 25th Annual Meeting of the National Sickle Cell Disease Program. 2001:Abstract no:#8.
  • Adams RJ, Brambilla DJ, McKie V, Files B, Vichinsky E, Abboud M, et al. Stroke prevention in sickle cell disease (STOP): final results [abstract]. Blood 2000;96(11 Pt 1):10a.
  • Adams RJ, Brambilla DJ, McKie V, Files B, Vichinsky E, Abboud M, et al. Stroke prevention in sickle cell disease (STOP): follow-up after closure of the trial [abstract]. Blood 1998;92(10 Suppl 1):527a.
  • Adams RJ, Brambilla DJ, McKie VC, Files B, Vichinsky E, Abboud M, et al. Stroke prevention in sickle cell disease (STOP): baseline characteristics of trial patients [abstract]. Blood 1997;90(10 Suppl 1):123a.
  • Adams RJ, Brambilla DJ, Vichinsky E, Abboud M, Pegelow C, Carl EM, et al. Risk of stroke and conversion to abnormal TCD in children screened with transcranial doppler (TCD) during the STOP study [abstract]. Conference Proceedings of the National Sickle Cell Disease Program 30th Annual Meeting; 2002 Sept 17-21. 2002:3.
  • Adams RJ, Brambilla DJ, Vichinsky E, Abboud M, Pegelow C, Carl EM, et al. Stroke prevention trial in sickle cell anemia (STOP study): Risk of stroke in 1933 children screened with transcranial doppler (TCD) [abstract]. Proceedings of the 23rd Annual Meeting of the National Sickle Cell Disease Program. 1999:54.
  • Adams RJ, Carl EM, McKie VC, Odo NA, Kutlar A, Phillips M, et al. A pilot trial of hydroxyurea to prevent strokes in children with sickle cell anemia [abstract]. Proceedings of the 23rd Annual Meeting of the National Sickle Cell Disease Program. 1999:53.
  • Adams RJ, McKie V, Files B, Vichinsky E, Abboud M, Hsu L, et al. Stroke prevention in sickle cell disease (STOP): Results of transcranial doppler ultrasound screening stroke risk [abstract]. Stroke 1998;29(1):308.
  • Adams RJ, McKie VC, Brambilla DJ, Carl E, Gallagher D, Nichols FT, et al. Stroke prevention trial in sickle cell anemia. Controlled Clinical Trials 1998;19(1):110-29.
  • Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial doppler ultrasonography. New England Journal of Medicine 1998;339(1):5-11.
  • Anonymous. Transfusion to prevent first stroke in children with sickle cell anemia. Journal of the American Ostoepathic Association 1998;98(7):363-4.
  • Bulas DI, Jones A, Seibert JJ, Driscoll C, O'Donnell R, Adams RJ. Transcranial Doppler (TCD) screening for stroke prevention in sickle cell anemia: pitfalls in technique variation. Pediatric Radiology 2000;30(11):733-8.
  • Cohen AR. Sickle cell disease - new treatments, new questions. New England Journal of Medicine 1998;339(1):42-4.
  • Duncan GW. Ischemic stroke in sickle cell disease: a review. Tennessee Medicine 1997;90(12):498-9.
  • Files B, Brambilla D, Kutlar A, Miller S, Pegelow C, Vichinsky E, et al. A randomized trial of chronic transfusion to prevent stroke in sickle cell anemia: changes in ferritin with chronic transfusion [abstract]. Blood 1998;92(10 Suppl 1):528a.
  • Files B, Brambilla D, Kutlar A, Miller S, Vichinsky E, Wang W, et al. Longitudinal changes in ferritin during chronic transfusion: a report from the Stroke Prevention Trial in Sickle Cell Anemia (STOP). Journal of Pediatric Hematology/Oncology 2002;24(4):284-90.
  • Gates A, Rogers MA, Puczynski M. Stroke prevention trial in sickle cell anemia: comments on effects of chronic transfusion on pain [letter]. Journal of Pediatrics 2002;141(5):742-3.
  • Hsu LL, Miller ST, Wright E, Kutlar A, McKie V, Wang W, et al. Alpha thalassemia is associated with decreased risk of abnormal transcranial doppler ultrasonography in children with sickle cell anemia. Journal of Pediatric Hematology/Oncology 2003;25(8):622-8.
  • Kutlar A, Brambilla D, Clair B, Haghighat A, Bakanay S, Adams G, et al. Candidate gene polymorphisms and their association with TCD velocities in children with sickle cell disease [abstract]. Blood 2007;110(11):Abstract no: 429.
  • Kutlar A, Harbin J, Jackson B, Holley L, Gallagher D, Clair B, et al. Laboratory parameters in patients randomized in the STOP study and their modification by transfusion [abstract]. Blood 2000;96(11 Pt 2):18b-9b.
  • Kutlar A, Harbin J, Jackson BB, Holley LGD, Clair B, Brambilla D, et al. Baseline laboratory parameters in patients randomized to the STOP study and their modification by transfusion [abstract]. Proceedings of the 24th Annual Meeting of the National Sickle Cell Disease Program. 2000:82a.
  • Kwiatkowski JL, Brambilla DJ, Granger S, Adams RJ. Elevated blood flow velocity in the anterior cerebral artery and stroke risk in sickle cell disease [abstract]. Blood 2003;102(11):Abstract no: 408.
  • Kwiatkowski JL, Granger S, Brambilla DJ, Brown RC, Miller ST, Adams RJ. Elevated blood flow velocity in the anterior cerebral artery and stroke risk in sickle cell disease: extended analysis from the STOP trial. British Journal of Haematology 2006;134(3):333-9.
  • Kwiatkowski JL, Morales K, Brambilla DJ, Files B, Adamkiewicz T, Adams RJ. Long-term follow-up of transcranial doppler ultrasonography in children with sickle cell disease: Results of the STOP and STOP II patient cohorts [abstract]. Blood 2002;100(11 Pt 1):663a.
  • Lee MT, Piomelli S, Granger S, Miller ST, Harkness S, Brambilla DJ, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood 2006;108(3):847-52.
  • Lee SB, Ramsingh D, Kutlar A, Holley L, McVie VC, Adams RJ. C-reactive protein in children with sickle cell disease at risk for stroke in the STOP study [abstract]. Stroke 2002;33(1):373.
  • Lezcano NE, Odo N, Kutlar A, Brambilla D, Adams RJ. Regular transfusion lowers plasma free hemoglobin in children with sickle-cell disease at risk for stroke. Stroke 2006;37(6):1424-6.
  • Miller ST, Wright E, Abboud M, Berman B, Files B, Scher C, et al. Impact of chronic transfusion on non-neurological events during the Stroke Prevention Trial in sickle cell anemia (STOP) [abstract]. Pediatric Research 2000;47(4 Pt 2):251A.
  • Miller ST, Wright E, Abboud M, Berman B, Files B, Scher C, et al. Impact of chronic transfusion on non-neurological events during the stroke prevention trial in sickle cell anemia (TOP) [abstract]. Proceedings of the 24th Annual Meeting of the National Sickle Cell Disease Program. 2000:61a.
  • Miller ST, Wright E, Abboud M, Berman B, Files B, Scher CD, et al. Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia. Journal of Pediatrics 2001;139(6):785-9.
  • Nichols FT, Jones AM, Adams RJ. Stroke prevention in sickle cell disease (STOP) study guidelines for transcranial Doppler testing. Journal of Neuroimaging 2001;11(4):354-62.
  • Olivieri N, Brambilla D, McKie V, Piomelli S, Kutlar A, Files B, et al. Changes in cerebral blood flow velocities during chronic transfusion therapy to prevent stroke in sickle cell disease [abstract]. Blood 2000;96(11 Pt 1):486a.
  • Pegelow CH, Adams R, Hsu L, McKie V, Wang W, Zimmerman R, et al. Children with silent infarct and elevated transcranial doppler ultrasonography velocity are at increased risk of subsequent infarctive events [abstract]. Proceedings of the 23rd Annual Meeting of the National Sickle Cell Disease Program. 1999:137.
  • Pegelow CH, Wang W, Granger S, Hsu LL, Vichinsky E, Moser FG, et al. Silent infarcts in children with sickle cell anemia and abnormal cerebral artery velocity. Archives of Neurology 2001;58(12):2017-21.
  • Styles L, De Jong K, Vichinsky E, Lubin B, Adams R, Kuypers F. Increased RBC phosphatidylserine exposure in sickle cell disease patients at risk for stroke by transcranial Doppler screening [abstract]. Blood 1997;90(10 Suppl 1):604a-5a.
  • Vichinsky E, Luban E, Wright E, Olivieri N, Driscoll C, Pegelow C, et al. Prospective red cell phenotype matching in STOP - a multi-centre transfusion trial [abstract]. Proceedings of the 23rd Annual Meeting of the National Sickle Cell Disease Program. 1999:163.
  • Vichinsky E, Luban N, Wright E, Olivieri N, Driscoll C, Pegelow C, et al. Prospective red cell phenotype matching in STOP - a multi-center transfusion trial [abstract]. Blood 1998;92(10 Suppl 1):528a.
  • Vichinsky EP, Luban NL, Wright E, Olivieri N, Driscoll C, Pegelow CH, et al. Prospective RBC phenotype matching in a stroke-prevention trial in sickle cell anemia: a multicenter transfusion trial. Transfusion 2001;41(9):1086-92.
  • Wang W, Morales K, Olivieri N, Styles L, Scher C, Adams R, et al. Effect of chronic transfusion on growth in children with sickle cell anemia: results of the STOP trial [abstract]. National Sickle Cell Disease Program 30th Annual Meeting Conference Proceedings; 2002 Sept 17-21. 2002:100.
  • Wang WC, Morales KH, Scher CD, Styles L, Olivieri N, Adams R, et al. Effect of long-term transfusion on growth in children with sickle cell anemia: results of the STOP trial. Journal of Pediatrics 2005;147(2):244-7.
STOP 2 2005 {published and unpublished data}
  • ASH News Daily 2004. NHLBI stops the STOP II trial early. www.hematology.org/publications/newsdaily/2004/issue_3/NHLBI.cfm (accessed 22 November 2005).
  • Abboud MR, Yim E, Adams RJ. The progression and development of silent infarcts in children with sickle cell anemia is prevented by chronic transfusions and is unrelated to level of hemolysis [abstract]. Blood 2008;12:Abstract no: 712.
  • Adamkiewicz T. Transcranial doppler measures in patients with sickle cell disease at high risk for stroke and receiving hydroxyurea: the HyRetro ancillary study [abstract]. Proceedings of the 52nd ASH Annual Meeting and Exposition; 2010 Dec 4-7; Orlando, Florida. 2010:Abstract no: 1620.
  • Adamkiewicz T, Abboud M, Barredo J, Cavalier ME, Peterson J, Rackoff B, et al. Serum Ferritin in children with SCD on chronic transfusion: correlation with serum alanine aminotransferase and liver iron (STOP/STOP II liver iron ancillary study) [abstract]. Proceedings of the 35th Annivesary Convention of the National Sickle Celll Disesae Program and the Siclkle Cell Disease Association of America; 2007 Sep 17-22; Washington DC, USA. 2007:316.
  • Adamkiewicz T, Abboud MR, Barredo JC, Kirby-Allen M, Alvarez OA, Casella JF, et al. Serum ferritin in children with sickle cell disease on chronic transfusion: measure of iron overload or end organ injury? STOP/ STOP II liver iron ancillary study [abstract]. Blood 2006;108(11):Abstract no: 791.
  • Adamkiewicz TV, Abboud MR, Paley C, Olivieri N, Kirby-Allen M, Vichinsky E, et al. Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury. Blood 2009;114(21):4632-8.
  • Adams RJ, Brambilla D. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. New England Journal of Medicine 2005;353(26):2769-78.
  • Adams RJ, Brambilla D, Miller ST. Optimizing primary stroke prevention in children with sickle cell [abstract]. 28th Annual Meeting of the National Sickle Cell Disease Program; 2005 April 9-13; Cincinnati, Ohio. 2005:3.
  • Adams RJ, Brambilla DJ, STOP II investigators. The study design of optimizing primary stroke prevention in children with sickle cell anemia [abstract]. 27th Annual Meeting of the National Sickle Cell Disease Program; 2004 April 18-21; Los Angeles, California. 2004:174.
  • Alvarez O, Miller S, Berman B, Brown C, Casella J, Coates T, et al. Evaluation of chronic transfusion practices in children with sickle cell disease: a survey of STOP II investigators. Blood 2004;104(11):Abstract no: 3732.
  • Brambilla DJ, Adams RJ. Stroke prevention in sickle cell anemia (STOP 2). National Institutes of Health, Clinical Trials Database Started in 06/2000.
  • Brown C, Miller S, Kwiatkowski J, Brambilla D, Adams R. Optimizing primary stroke prevention in sickle cell anemia (STOP 2): an argument for prolonged transfusion? [abstract]. 29th Annual Meeting of the National Sickle Cell Disease Program; 2006 April 8-12; Memphis, USA.. 2006:78.
  • Kutlar A, Brambilla D, Clair B, Haghighat A, Bakanay S, Adams G, et al. Candidate gene polymorphisms and their association with TCD velocities in children with sickle cell disease [abstract]. Blood 2007;110(11):Abstract no: 429.
  • Kwiatkowski JL, Morales K, Brambilla DJ, Files B, Adamkiewicz T, Adams RJ, et al. Long-term follow-up of transcranial doppler ultrasonography in children with sickle cell disease: results of the STOP and STOP II patient cohorts. Blood 2002;100(11 Pt 1):663a.
  • Medical College of Georgia. STOP II Questions and Answers. www.mcg.edu/neurology/Research/sicklecell/STOPIIQandA.htm (accessed 22 November 2005).
  • Medical News Today. NHLBI stops sickle cell anemia transfusion study. www.medicalnewstoday.com/printerfriendlynews.php?newsid=17363 (accessed 22 November 2005).
SWiTCH 2012 {published data only}
  • Aygun B, Mortier NA, Kesler K, Schultz WH, Alvarez OA, Rogers ZR, et al. Therapeutice phlebotomy in children with sickle cell anemia, stroke, and iron overload: the SWiTCH experience [abstract]. Procedings of the 53rd ASH Annual Meeting and Exposition; 2011 Dec10-13; San Diego, California. 2011:Abstract no: 1044.
  • NCT00122980. Stroke with transfusions changing to hydroxyurea (SWiTCH). www.clinicaltrials.gov/show/NCT00122980 (accessed 16th August 2011).
  • Ware RE, Helms RW. Stroke with transfusions changing to hydroxyurea (SWiTCH). Blood 2012; Vol. 119, issue 17:3925-32.
  • Ware RE, Helms RW. Stroke with transfusions changing to hydroxyurea (SWiTCH): a phase 3 randomised clinical trial for treatment of children with sickle cell anemia [abstract]. Proceedings of the 52nd ASH Meeting and Exposition; 2010 Dec 4-7; Orlando, Florida. 2010:Abstract no: 844.
  • Ware RE, Helms RW, SWiTCH Investigators. Stroke with transfusions changing to hydroxyurea (SWiTCH). Blood 2012;119(17):3925-32.
  • Ware RE, McMurray MA, Schultz WH, Alvarez OA, Aygun B, Cavalier ME, et al. Academic community standards for chronic transfusion therapy in children with sickle cell anemia and stroke [abstract]. Blood 2006;108(11):Abstract no: 1213.
  • Ware RE, Schultz WH, Yovetich N, Mortier NA, Alvarez O, Hilliard L, et al. Stroke with transfusions changing to hydroxyurea (SWiTCH): A phase III randomized clinical trial for treatment of children with sickle cell anemia, stroke, and iron overload. Pediatric Blood & Cancer 2011;57(6):1011-7.

References to ongoing studies

  1. References to studies included in this review
  2. References to ongoing studies
  3. Additional references
  4. References to other published versions of this review
SCATE Trial {published data only}
  • NCT01531387. Sparing Conversion to Abnormal TCD (Transcranial Doppler) Elevation (SCATE). http://clinicaltrials.gov/show/NCT01531387 (accessed 06 November 2013).
SIT Trial {published data only}
  • Bhatnagar P, Purvis S, Barron-Casella E, DeBaun MR, Casella JF, Arking DE, et al. Genome-wide association study identifies genetic variants influencing F-cell levels in sickle cell patients. Journal of Human Genetics 2011;56(4):316-23.
  • Bhatnagar P, Purvis S, Barron-Casella E, DeBaun MR, Casella JF, Arking DE, et al. Online Supplementary Information to 'Genome-wide association study identifies genetic variants influencing F-cell levels in sickle-cell patients' [online]. Journal of Human Genetics 2011;56(4):316-23.
  • Casella JF, King AA, Barton B, White DA, Noetzel MJ, Ichord RN, et al. Design of the silent cerebral infarct transfusion (SIT) trial. Pediatric Hematology and Oncology 2010;27(2):69-89.
  • DeBaun M. Epidemiology and treatment of silent strokes in sickle cell anemia [abstract]. 28th Annual Meeting of the National Sickle Cell Disease Program; 2005 April 9-13; Cincinnati, Ohio. 2005:Plenary.
  • Jordan LC, McKinstry RC 3rd, Kraut MA, Ball WS, Vendt BA, Casella JF, et al. Incidental findings on brain magnetic resonance imaging of children with sickle cell disease. Pediatrics 2010;126(1):53-61.
  • Vendt BA, McKinstry RC, Ball WS, Kraut MA, Prior FW, Barton B, et al. Silent Cerebral Infarct Transfusion (SIT) trial imaging core: application of novel imaging information technology for rapid and central review of MRI of the brain. Journal of Digital Imaging 2009;22(3):326-43.
TWiTCH Trial {published data only}
  • NCT01425307. Transcranial Doppler (TCD) With Transfusions Changing to Hydroxyurea (TWiTCH). http://clinicaltrials.gov/show/NCT01425307 (accessed 06 November 2013).

Additional references

  1. References to studies included in this review
  2. References to ongoing studies
  3. Additional references
  4. References to other published versions of this review
Adams 1992
Adams 1996
  • Adams DM, Schultz WH, Ware RE, Kinney TR. Erythrocytapheresis can reduce iron overload and prevent the need for chelation therapy in chronically transfused pediatric patients. Journal of Pediatric Hematology Oncology 1996;18(1):46-50.
Adams 1998a
Adams 1998b
  • Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. New England Journal of Medicine 1998;339(1):5-11.
Aygun 2009
Bernaudin 2011
  • Bernaudin F, Verlhac S, Arnaud C, Kamdem A, Chevret S, Hau I, et al. Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anemia cohort. Blood 2011;117(4):1130-40.
Brousseau 2010
  • Brousseau DC, Panepinto JA, Nimmer M, Hoffmann RG. The number of people with sickle-cell disease in the United States: national and state estimates. American Journal of Hematology 2010;85(1):77-8.
Casella 2010
Cohen 1992
  • Cohen AR, Martin MB, Silber JH, Kim HC, Ohene-Frempong K, Schwarz E. A modified program for prevention of stroke in sickle cell disease. Blood 1992;79(7):1657-61.
Cohen 1996
  • Cohen AR, Norris CF, Smith-Whitley K. Transfusion therapy for sickle cell disease. In: Capon SM, Chambers LA editor(s). New Directions in Pediatric Hematology. Bethesda MD: American Association of Blood Banks, 1996:39-85.
Davies 1995
DeBaun 2006
Enninful-Eghan 2010
  • Enninful-Eghan H, Moore RH, Ichord R, Smith-Whitley K, Kwiatkowski JL. Transcranial doppler ultrasonography and prophylactic transfusion program is effective in preventing overt stroke in children with sickle cell disease. Journal of Pediatrics 2010;157(3):479-84.
Fullerton 2004
Gulbis 2005
  • Gulbis B,  Haberman D,  Dufour D,  Christophe C,  Vermylen C,  Kagambega F, et al. Hydroxyurea for sickle cell disease in children and for prevention of cerebrovascular events: the Belgian experience. Blood 2005;105(7):2685-90.
Hassell 2010
Hickman 1999
Hulbert 2011
  • Hulbert ML, McKinstry RC, Lacey JL, Moran CJ, Panepinto JA, Thompson AA, et al. Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease. Blood 2011;117(3):772-9.
Inati 2011
Karnon 2008
  • Karnon J, Tolley K, Oyee J, Jewitt K, Ossa D, Akehurst R. Cost-utility analysis of deferasirox compared to standard therapy with desferrioxamine for patients requiring iron chelation therapy in the United Kingdom. Current Medical Research and Opinion 2008;24(6):1609-21.
Kinney 1999
Kratovil 2006
Kwiatkowski 2009
Lee 2006
  • Lee MT, Piomelli S, Granger S, Miller ST, Harkness S, Brambilla DJ, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood 2006;108(3):847-52.
McCarville 2008
Miller 2001
  • Miller ST, Wright E, Abboud M, Berman B, Files B, Scher CD, et al. Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia. Journal of Pediatrics 2001;139(6):785-9.
Nagel 2003
Ohene-Frempong 1999
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Pegelow 1995
  • Pegelow CH, Adams RJ, McKie V, Abboud M, Berman B, Miller ST, et al. Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. Journal of Pediatrics 1995;126(6):896-9.
Pegelow 2001
  • Pegelow CH, Wang W, Granger S, Hsu LL, Vichinsky E, Moser FG, et al. Silent infarcts in children with sickle cell anemia and abnormal cerebral artery velocity. Archives of Neurology 2001;58(12):2017-21.
Platt 1994
  • Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, et al. Mortality in sickle cell disease: life expectancy and risk factors for early death. New England Journal of Medicine 1994;330(23):1639-44.
Powars 2000
Quinn 2010
Rana 1997
  • Rana S, Houston PE, Surana N, Shalaby-Rana EI, Castro OL. Discontinuation of long-term transfusion therapy in patients with sickle cell disease and stroke. Journal of Pediatrics 1997;131(5):757-60.
Sarnaik 1979
  • Sarnaik S, Soorya D, Kim J, Ravindranath Y, Lusher J. Periodic transfusions for sickle cell anemia and CNS infarction. American Journal of Diseases of Children 1979;133(12):1254-7.
Scothorn 2002
  • Scothorn DJ,  Price C,  Schwartz D,  Terrill C,  Buchanan GR,  Shurney W,  et al. Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke. Journal of Pediatrics 2002;140(3):48-54.
Serjeant 1992
  • Serjeant GR. Surgery and anaesthesia. Sickle Cell Disease. 2nd Edition. Oxford: Oxford University Press, 1992:455-8.
Smith-Whitley 2012
Steinberg 1999
Styles 1994
Telfer 2007
  • Telfer P, Coen P, Chakravorty S, Wilkey O, Evans J, Newell H, et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica 2007;92(7):905-12.
Venkatesubramanian 1994
Wang 1991
  • Wang W, Kovnar EH, Tonkin IL, Mulhern RK, Langston JW, Day S, et al. High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease. Journal of Pediatrics 1991;118(3):377-82.
Wang 2005
  • Wang WC, Morales KH, Scher CD, Styles L, Olivieri N, Adams R, et al. Effect of long-term transfusion on growth in children with sickle cell anemia: results of the STOP trial. Journal of Pediatrics 2005;147(2):244-7.
Ware 2004
  • Ware RE, Zimmermann SA, Sylvestre PB, Mortier NA, Davis JS, Treem WR, et al. Prevention of secondary stroke and resolution of transfusional iron overload in children with sickle cell anemia using hydroxyurea and phlebotomy. Journal of Pediatrics 2004;145(3):346-52.
Ware 2010
Wayne 2000
Wilimas 1980
  • Wilimas J, Goff JR, Anderson HR, Langston JW, Thomson E. Efficacy of transfusion therapy for one to two years in patients with sickle cell disease and cerebrovascular accidents. Journal of Pediatrics 1980;96(2):205-8.
Zimmerman 2007
  • Zimmerman SA,  Schultz WH,  Burgett S,  Mortier NA,  Ware RE. Hydroxyurea therapy lowers transcranial Doppler flow velocities in children with sickle cell anemia. Blood 2007;110(3):1043-7.

References to other published versions of this review

  1. References to studies included in this review
  2. References to ongoing studies
  3. Additional references
  4. References to other published versions of this review
Hirst 2002
  • Hirst C, Wang WC. Blood transfusion for preventing stroke in people with sickle cell disease. Cochrane Database of Systematic Reviews 2002, Issue 1. [DOI: 10.1002/14651858.CD003146]