Skip to main content
SpringerLink
Log in

Does regular blood transfusion prevent progression of cerebrovascular lesions in children with sickle cell disease?

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

A retrospective study was conducted to assess changes in cerebrovascular lesions, as assessed by magnetic resonance (MR) imaging and angiography in 18 children with sickle cell disease (SCD) receiving optimised chronic transfusions for primary stroke prevention (abnormal transcranial Doppler flow, nine patients, median follow-up 14.3 months (range, 7.9–48.9)) or secondary stroke prevention (nine patients, median follow-up 59.6 months (range, 11.0–127.9)). An experienced neuroradiologist blinded to patient data reviewed the 41 MR scans (median/patient, three (2–4)). Standard scores were used to evaluate parenchymal and vascular abnormalities at baseline and last follow-up. Within-patient score changes evaluated using Wilcoxon’s paired rank test indicated lesion progression in the secondary-prevention group (p = 0.027). Optimised transfusion therapy does not prevent progression of cerebral vasculopathy in SCD children with a history of stroke.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Steen RG, Emudianughe T, Hankins GM, Wynn LW, Wang WC, Xiong X, Helton KJ (2003) Brain imaging findings in pediatric patients with sickle cell disease. Radiology 228:216–225. doi:10.1148/radiol.2281020943

    Article  PubMed  Google Scholar 

  2. Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury S, Moohr JW, Wethers DL, Pegelow CH, Gill FM (1998) Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood 91:288–294

    PubMed  CAS  Google Scholar 

  3. Steen RG, Miles MA, Helton KJ, Strawn S, Wang W, Xiong X, Mulhern RK (2003) Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit. AJNR Am J Neuroradiol 24:382–389

    PubMed  Google Scholar 

  4. Wang W, Enos L, Gallagher D, Thompson R, Guarini L, Vichinsky E, Wright E, Zimmerman R, Armstrong FD (2001) Neuropsychologic performance in school-aged children with sickle cell disease: a report from the Cooperative Study of Sickle Cell Disease. J Pediatr 139:391–397. doi:10.1067/mpd.2001.116935

    Article  PubMed  CAS  Google Scholar 

  5. Bernaudin F, Verlhac S, Freard F, Roudot-Thoraval F, Benkerrou M, Thuret I, Mardini R, Vannier JP, Ploix E, Romero M, Casse-Perrot C, Helly M, Gillard E, Sebag G, Kchouk H, Pracros JP, Finck B, Dacher JN, Ickowicz V, Raybaud C, Poncet M, Lesprit E, Reinert PH, Brugieres P (2000) Multicenter prospective study of children with sickle cell disease: radiographic and psychometric correlation. J Child Neurol 15:333–343. doi:10.1177/088307380001500510

    Article  PubMed  CAS  Google Scholar 

  6. Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, Abboud M, Gallagher D, Kutlar A, Nichols FT, Bonds DR, Brambilla D (1998) Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med 339:5–11. doi:10.1056/NEJM199807023390102

    Article  PubMed  CAS  Google Scholar 

  7. Pegelow CH, Adams RJ, McKie V, Abboud M, Berman B, Miller ST, Olivieri N, Vichinsky E, Wang W, Brambilla D (1995) Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. J Pediatr 126:896–899. doi:10.1016/S0022-3476(95)70204-0

    Article  PubMed  CAS  Google Scholar 

  8. Scothorn DJ, Price C, Schwartz D et al (2002) Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke. J Pediatr 140:348–354. doi:10.1067/mpd.2002.122498

    Article  PubMed  Google Scholar 

  9. Wang WC, Kovnar EH, Tonkin IL et al (1991) High risk of recurrent stroke after discontinuance of five to twelve years of transfusiont therapy in patients with sickle cell disease. J Pediatr 118:377–382. doi:10.1016/S0022-3476(05)82150-X

    Article  PubMed  CAS  Google Scholar 

  10. Adams RJ, Brambilla D (2005) Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med 353:2769–2778. doi:10.1056/NEJMoa050460

    Article  PubMed  CAS  Google Scholar 

  11. Venketasubramanian N, Prohovnik I, Hurlet A, Mohr JP, Piomelli S (1994) Middle cerebral artery velocity changes during transfusion in sickle cell anemia. Stroke 25:2153–2158

    PubMed  CAS  Google Scholar 

  12. Russell MO, Goldberg HI, Hodson A, Kim HC, Halus J, Reivich M, Schwartz E (1984) Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease. Blood 63:162–169

    PubMed  CAS  Google Scholar 

  13. Woodard P, Helton KJ, Khan RB et al (2005) Brain parenchymal damage after haematopoietic stem cell transplantation for severe sickle cell disease. Br J Haematol 129:550–552. doi:10.1111/j.1365-2141.2005.05491.x

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Reference Centre for Haemoglobinopathies, Hôpital Necker, APHP, Paris, France

    Valentine Brousse & Mariane de Montalembert

  2. Paediatrics Department, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015, Paris, France

    Valentine Brousse, Elsa Mirre, Gérard Lenoir & Mariane de Montalembert

  3. Department of Paediatric Radiology, Hôpital Necker, APHP, Paris, France

    Lucie Hertz-Pannier

  4. INSERM U663, Paris Descartes University, Paris, France

    Lucie Hertz-Pannier

  5. Department of Hepatology, Hôpital Beaujon, APHP, Paris, France

    Yann Consigny

  6. Clinical Research Unit, Hôpital Necker, APHP, Paris, France

    Jean-Louis Bresson

  7. Haematology Laboratory, Hôpital Tenon, APHP, Paris, France

    Robert Girot

Authors
  1. Valentine Brousse
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lucie Hertz-Pannier
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Yann Consigny
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jean-Louis Bresson
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Robert Girot
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Elsa Mirre
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Gérard Lenoir
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Mariane de Montalembert
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Valentine Brousse.

Additional information

Financial support: No financial assistance was received in support of this study.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Brousse, V., Hertz-Pannier, L., Consigny, Y. et al. Does regular blood transfusion prevent progression of cerebrovascular lesions in children with sickle cell disease?. Ann Hematol 88, 785–788 (2009). https://doi.org/10.1007/s00277-008-0670-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-008-0670-x

Keywords

Search

Navigation