Blood Reviews
Volume 23 , Pages S9-S13 , December 2009

Recent advances in improving the management of sickle cell disease

  • Adlette Inati

      Affiliations

    • Corresponding Author InformationCorrespondence: Dr. Adlette Inati. Head, Division of Pediatric Hematology-Oncology, Medical Director, Children's Center for Cancer and Blood Diseases, Rafik Hariri University Hospital, Beirut, Lebanon. Research Associate, Balamand University, Consultant Hematologist, Chronic Care Center, Beirut, Beirut, Lebanon

References 

  1. Inati A , Koussa S , Taher A , et al.   Sickle cell disease: new insights into pathophysiology and treatment . Pediatr Ann . 2008;37:311–325
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  8. Adams RJ , McKie VC , Hsu L , et al.   Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography . N Engl J Med . 1998;339:5–11
  9. Vichinsky EP , Haberkern CM , Neumayr L , et al.   A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group . N Engl J Med . 1995;333:206–213
  10. Ohene-Frempong K . Indications for red cell transfusion in sickle cell disease . Semin Hematol . 2001;38(1 Suppl 1):5–13
  11. Pegelow CH , Adams RJ , McKie V , et al.   Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions . J Pediatr . 1995;126:896–899
  12. Adams RJ , Brambilla D . Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) Trial Investigators. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease . N Engl J Med . 2005;353:2769–2778
  13. Al-Refaie FN , Wickens DG , Wonke B , et al.   Serum non-transferrin bound iron in beta-thalassaemia major patients treated with desferrioxamine and L1 . Br J Haematol . 1992;82:431–436
  14. Darbari DS , Kple-Faget P , Kwagyan J , et al.   Circumstances of death in adult sickle cell disease patients . Am J Haematol . 2006;81:858–863
  15. Aduloju SO , Palmer S , Eckman JR . Mortality in sickle cell patient transitioning from pediatric to adult program: 10 years grady comprehensive sickle cell center experience . Blood . 2008;112: abstract 1426.
  16. Fung EB , Harmatz PR , Milet M , et al.   Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions . Transfusion . 2008;48:1971–1980
  17. Cappellini MD , Porter J , El-Beshlawy A , et al.   Tailoring iron chelation by iron intake and serum ferritin: prospective EPIC study of deferasirox in 1744 patients with transfusion-dependent anemias . Haematologica . 2009; [Epub ahead of print].
  18. Breuer W , Hershko C , Cabantchik ZI . The importance of non-transferrin bound iron in disorders of iron metabolism . Transfus Sci . 2000;23:185–192
  19. Inati A , Taha M , Musallam KM , et al.   Magnetic resonance imaging R2 mapping accurately estimates iron overload in sickle cell disease patients . EHA . 2009; abstract 1285.
  20. Inati A, Musallam KM, Wood JC, et al. Iron overload indices rise linearly with transfusion rate in patients with sickle cell disease. Blood; in press.
  21. Inati A , Musallam KM , Wood JC , et al.   Absence of cardiac siderosis by MRI T2* despite transfusion burden, hepatic and serum iron overload in Lebanese patients with sickle cell disease . Eur J Haematol . 2009;83:565–571
  22. Inati A , Musallam KM , Taha M , et al.   Magnetic resonance imaging T2* in the evaluation of cardiac iron overload in patients with sickle cell disease . EHA . 2009; abstract 1286.
  23. Sickle Cell Society, UK, 2008 . Available at: www.sicklecellsociety.org/CareBook.pdf
  24. National Institutes of Health  , National Heart, Lung, and Blood Institute, and Division of Blood Diseases and Resources  . The management of sickle cell disease (NIH Publication No 02-2117) . Bethesda, MD: NIH; 2002;
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  26. Vichinsky E , Pakbaz Z , Onyekwere O , et al.   Patient-reported outcomes of deferasirox (Exjade, ICL670) versus deferoxamine in sickle cell disease patients with transfusional hemosiderosis. Substudy of a randomized open-label phase II trial . Acta Haematol . 2008;119:133–141
  27. Vichinsky E. Coates T, Thompson AA, et al. Deferasirox (Exjade®), the once-daily oral iron chelator, demonstrates safety and efficacy in patient with sickle cell disease (CD: 3.5-year follow-up. ASH 2008: abstract 1420.
  28. Raphael JL , Bernhardt MB , Mahoney DH , et al.   Oral iron chelation and the treatment of iron overload in a pediatric hematology center . Pediatr Blood Cancer . 2009;52:616–620

PII: S0268-960X(09)70004-9

doi: 10.1016/S0268-960X(09)70004-9

Blood Reviews
Volume 23 , Pages S9-S13 , December 2009

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