Current Understanding in the Management of Sickle Cell Disease

REFERENCES

• Herrick JB. Peculiar elongated and sickled-shaped red blood corpuscles in a case of severe anemia. Arch Intern Med. 1910;6:517–521.
   Google Scholar
• Pauling L, Itano HA, Singer SJ, Wells IC. Sickle cell anemia: a molecular disease. Science. 1949;110:543–548.
   PubMed Web of Science ®Google Scholar
• Ingram VM. Gene mutations in human haemoglobin: the chemical difference between normal and sickle haemoglobin. Nature. 1957;180:326–328.
   PubMed Web of Science ®Google Scholar
• Perutz MF, Rossmann MG, Cullis AF, et al. Structure of haemoglobin: a three-dimensional Fourier synthesis at 5·5Å resolution obtained by X-ray analysis. Nature. 1960;185:416–422.
   PubMed Web of Science ®Google Scholar
• Watson J, Stahman AW, Billelo FP. The significance of the paucity of sickle cells in newborn Negro infants. Am J Med Sci. 1948;215:419–423.
   PubMed Web of Science ®Google Scholar
• Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. 2004;364:1343–1360.
   PubMed Web of Science ®Google Scholar
• Inati A, Koussa S, Taher A, Perrine S. Sickle cell disease: new insights into pathophysiology and treatment. Pediatr Ann. 2008;37(5):311–321.
   PubMedGoogle Scholar
• Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med. 1994;330:1639–1644.
   PubMed Web of Science ®Google Scholar
• Inati A, Taher A, Bou Alawi W, et al. Beta-globin gene cluster haplotypes and HbF levels are not the only modulators of sickle cell disease in Lebanon. Eur J Haematol. 2003;70:79–83.
   PubMed Web of Science ®Google Scholar
• Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial. N Engl J Med. 1986;314:1593–1599.
   PubMed Web of Science ®Google Scholar
• Falletta JM, Woods GM, Verter JI, et al. Discontinuing penicillin prophylaxis in children with sickle cell anemia. Prophylactic penicillin study II. J Pediatr. 1995;127:685–690.
   PubMed Web of Science ®Google Scholar
• 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(4):206–213.
   PubMed Web of Science ®Google Scholar
• Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on frequency of painful crises in sickle cell anemia. Investigators of the multicenter study of hydroxyurea in sickle cell anemia. N Engl J Med. 1995;332(2):1317–1322.
   PubMedGoogle Scholar
• Steinberg MH, Barton F, Castro O, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. J Am Med Assoc. 2003;289(13):1645–1651.
   PubMed Web of Science ®Google Scholar
• Kinney TR, Helms RW, O'Branski EE, et al. Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial. Pediatric hydroxyurea group. Blood. 1999;94(5):1550–1554.
   PubMed Web of Science ®Google Scholar
• Hankins JS, Ware RE, Rogers ZR, et al. Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study. Blood. 2005;106(7):2269–2275.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Aygun B, McMurray MA, Schultz WH, et al. Chronic transfusion practice for children with sickle cell anaemia and stroke. Br J Haematol. 2009;145(4):524–528.
   PubMed Web of Science ®Google Scholar
• Zimmerman SA, Schultz WH, Davis JS, et al. Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerated dose in children with sickle cell disease. Blood. 2004;103(6):2039–2045.
   PubMed Web of Science ®Google Scholar
• 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):1043–1047.
   PubMed Web of Science ®Google Scholar
• Sickle Cell Society. Standards for the Clinical Care of Adults with Sickle Cell Disease in the UK. London: Sickle Cell Society; 2008.
   Google Scholar
• Ballas SK. Iron overload is a determinant of morbidity and mortality in adult patients with sickle cell disease. Semin Hematol. 2001;38 (1 Suppl. 1):30–36.
   PubMed Web of Science ®Google Scholar
• Olivieri NF. Progression of iron overload in sickle cell disease. Semin Hematol. 2001;38(1 Suppl. 1):57–62.
   PubMed Web of Science ®Google Scholar
• Cohen AR, Martin MB. Iron chelation therapy in sickle cell disease. Semin Hematol. 2001;38(1 Suppl. 1):69–72.
   Google Scholar
• Inati A, Taha M, Musallam K, Ziade F, Rachkidi Y, Taher A. Magnetic resonance imaging R2 mapping accurately estimates iron overload in sickle cell disease patients. Haematologica. 2009;94(Suppl. 2):511. Abs. 1285.
   Google Scholar
• Elliott V, Ellen B, Ellen F, et al. Comparison of organ dysfunction in transfused patients with SCD or beta thalassemia. Am J Hematol. 2005;80:70–74.
   Google Scholar
• Vichinsky E, Onyekwere O, Porter J, et al. A randomised comparison of deferasirox versus deferoxamine for the treatment of transfusional iron overload in sickle cell disease. Br J Haematol. 2007;136(3):501–508.
   PubMed Web of Science ®Google Scholar
• Voskaridou E, Douskou M, Terpos E, et al. Deferiprone as an oral iron chelator in sickle cell disease. Ann Hematol. 2005;84(7):434–440.
   PubMed Web of Science ®Google Scholar
• Walters MC, Storb R, Patience M, et al. Impact of bone marrow transplantation for symptomatic sickle cell disease: an interim report. Multicenter investigation of bone marrow transplantation for sickle cell disease. Blood. 2000;95(6):1918–1924.
   PubMed Web of Science ®Google Scholar
• Bernaudin F, Socie G, Kuentz M, et al. Long-term results of related myeloablative stem-cell transplantation to cure sickle cell disease. Blood. 2007;110(7):2749–2756.
   PubMed Web of Science ®Google Scholar
• Panepinto JA, Walters MC, Carreras J, et al. Matched-related donor transplantation for sickle cell disease: report from the Center for International Blood and Transplant Research. Br J Haematol. 2007;137(5):479–485.
   PubMed Web of Science ®Google Scholar
• Quinn CT, Rogers ZR, Buchanan GR. Survival of children with sickle cell disease. Blood. 2004;103(11):4023–4027.
   PubMed Web of Science ®Google Scholar
• Telfer P, Coen P, Chakravorty S, et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica. 2007;92(7):905–912.
   PubMed Web of Science ®Google Scholar