Coinheritance of α-Thalassemia Decreases the Risk of Cerebrovascular Disease in a Cohort of Children with Sickle Cell Anemia

REFERENCES

• Cançado RD, Jesus JA. A doença falciforme no Brasil. Rev Bras Hematol Hemoter. 2007;29(3):203–206.
   Google Scholar
• Fernandes AP, Januario JN, Cangussu CB, de Macedo DL, Viana MB. Mortality of children with sickle cell disease: a population study. J Pediatr (Rio J). 2010;86(4):279–284.
   PubMed Web of Science ®Google Scholar
• Rund D, Fucharoen S. Genetic modifiers in hemoglobinopathies. Curr Mol Med. 2008;(7):600–608.
   PubMedGoogle Scholar
• Steinberg MH. Predicting clinical severity in sickle cell anaemia. Br J Haematol. 2005;129(4):465–481.
   PubMed Web of Science ®Google Scholar
• Ballas SK. Effect of α-globin genotype on the pathophysiology of sickle cell disease. Pediatr Pathol Mol Med. 2001;20(2):107–121.
   PubMedGoogle Scholar
• Higgs DR, Aldridge BE, Lamb J, The interaction of α-thalassemia and homozygous sickle-cell disease. N Engl J Med. 1982;306(24):1441–1446.
   PubMed Web of Science ®Google Scholar
• Felice AE, McKie KM, Cleek MP, Marino EM, Kutlar A, McKie VC. Effects of α-thalassemia-2 on the developmental changes of hematological values in children with sickle cell disease from Georgia. Am J Hematol. 1987;25(4):389–400.
   PubMedGoogle Scholar
• Mouele R, Pambou O, Feingold J, Galacteros F. α-Thalassemia in Bantu population from Congo-Brazzaville: its interaction with sickle cell anemia. Hum Hered. 2000;50(2):118–125.
   PubMed Web of Science ®Google Scholar
• Adorno EV, Zanette A, Lyra I, Seixas MO, Reis MG, Gonçalves MS. Clinical and molecular characteristics of sickle cell anemia in the northeast of Brazil. Genet Mol Biol. 2008;31(3):621–625.
   Web of Science ®Google Scholar
• Bernaudin F, Verlhac S, Chevret S, G6PD deficiency, absence of α-thalassemia, and hemolytic rate at baseline are significant independent risk factors for abnormally high cerebral velocities in patients with sickle cell anemia. Blood. 2008;112(10):4314–4317.
   PubMed Web of Science ®Google Scholar
• Steinberg MH, Hsu H, Nagel RL, Gender and haplotype effects upon hematological manifestations of adult sickle cell anemia. Am J Hematol. 1995;48(3):175–181.
   PubMed Web of Science ®Google Scholar
• Tarer V, Etienne-Julan M, Diara JP, Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events. Eur J Haematol. 2006;76(3):193–199.
   PubMedGoogle Scholar
• Mouele R, Boukila V, Fourcade V, Feingold J, Galacteros F. Sickle-cell disease in Brazzaville, Congo: genetical, hematological, biochemical and clinical aspects. Acta Haematol. 1999;101(4):178–184.
   PubMedGoogle Scholar
• Embury SH, Clark MR, Monroy G, Mohandas N. Concurrent sickle cell anemia and α-thalassemia. Effect on pathological properties of sickle erythrocytes. J Clin Invest. 1984;73(1):116–123.
   PubMedGoogle Scholar
• Mukherjee MB, Surve R, Tamankar A, The influence of α-thalassaemia on the haematological and clinical expression of sickle cell disease in western India. Indian J Med Res. 1998;107(April):178–181.
   PubMedGoogle Scholar
• Neonato MG, Guilloud-Bataille M, Beauvais P, Acute clinical events in 299 homozygous sickle cell patients living in France. French Study Group on Sickle Cell Disease. Eur J Haematol. 2000;65(3):155–164.
   PubMedGoogle Scholar
• Adams RJ, Kutlar A, McKie V, α Thalassemia and stroke risk in sickle cell anemia. Am J Hematol. 1994;45(4):279–282.
   PubMed Web of Science ®Google Scholar
• Gill FM, Sleeper LA, Weiner SJ, Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease. Blood. 1995;86(11):776–783.
   PubMedGoogle Scholar
• Ohene-Frempong K, Weiner SJ, Sleeper LA, Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288–294.
   PubMed Web of Science ®Google Scholar
• Sarnaik SA, Ballas SK. Molecular characteristics of pediatric patients with sickle cell anemia and stroke. Am J Hematol. 2001;67(3):179–182.
   PubMedGoogle Scholar
• Koshy M, Entsuah R, Koranda A, Leg ulcers in patients with sickle cell disease. Blood. 1989;74(4):1403–1408.
   PubMed Web of Science ®Google Scholar
• Adekile AD, Tuli M, Haider MZ, Al-Zaabi K, Mohannadi S, Owunwanne A. Influence of α-thalassemia trait on spleen function in sickle cell anemia patients with high Hb F. Am J Hematol. 1996;53(1):1–5.
   PubMed Web of Science ®Google Scholar
• Wali YA, Al-Lamki Z, Hussein SS, Splenic function in Omani children with sickle cell disease: correlation with severity index, hemoglobin phenotype, iron status, and α-thalassemia trait. Pediatr Hematol Oncol. 2002;19(7):491–500.
   PubMed Web of Science ®Google Scholar
• Thomas PW, Higgs DR, Serjeant GR. Benign clinical course in homozygous sickle cell disease: a search for predictors. J Clin Epidemiol. 1997;50(2):121–126.
   PubMed Web of Science ®Google Scholar
• Milner PF, Kraus AP, Sebes JI, Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991;325(21):1476–1481.
   PubMed Web of Science ®Google Scholar
• Martinez G, Muniz A, Svarch E, Espinosa E, Nagel RL. Age dependence of the gene frequency of α-thalassemia in sickle cell anemia in Cuba. Blood. 1996;88(5):1898–1899.
   PubMedGoogle Scholar
• Miller ST, Sleeper LA, Pegelow CH, Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med. 2000;342(2):83–89.
   PubMed Web of Science ®Google Scholar
• Belisário AR, Martins ML, Brito AMS, Rodrigues CV, Silva CM, Viana MB. β-Globin gene cluster haplotypes in a cohort of 221 children with sickle cell anemia or Sβ0-thalassemia and its association with clinical and hematological features. Acta Haematol. 2010;124(3):162–170.
   PubMedGoogle Scholar
• Chang JC, Kan YW. Antenatal diagnosis of sickle cell anaemia by direct analysis of the sickle mutation. Lancet. 1981;2(8256):1127–1129.
   PubMed Web of Science ®Google Scholar
• Tan AS, Quah TC, Low PS, Chong SS. A rapid and reliable 7-deletion multiplex polymerase chain reaction assay for α-thalassemia. Blood. 2001;98(1):250–251.
   PubMed Web of Science ®Google Scholar
• Silva CM, Giovani P, Viana MB. High reticulocyte count is an independent risk factor for cerebrovascular disease in children with sickle cell anemia. Pediatr Blood Cancer. 2010; (in press) DOI 10.1002/pbc.22680.
   Google Scholar
• Adams RJ, McKie VC, Hsu L, 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(1):5–11.
   PubMed Web of Science ®Google Scholar
• Kwiatkowski JL, Zimmerman R, Greenbaum B, Ohene-Frempong K. Stroke and elevated blood flow velocity in the anterior cerebral artery in sickle cell disease. J Pediatr Hematol Oncol. 2004;26(5):323–326.
   PubMedGoogle Scholar
• Hsu LL, Miller ST, Wright E, α Thalassemia is associated with decreased risk of abnormal transcranial Doppler ultrasonography in children with sickle cell anemia. J Pediatr Hematol Oncol. 2003;25(8):622–628.
   PubMed Web of Science ®Google Scholar
• Hankins JS, Fortner GL, McCarville MB, The natural history of conditional transcranial Doppler flow velocities in children with sickle cell anaemia. Br J Haematol. 2008;142(1):94–99.
   PubMedGoogle Scholar
• Rezende PV, Viana MB, Murao M, Chaves AC, Ribeiro AC. Acute splenic sequestration in a cohort of children with sickle cell anemia. J Pediatr (Rio J). 2009;85(2):163–169.
   PubMedGoogle Scholar
• Lyra IM, Goncalves MS, Braga JA, Clinical, hematological, and molecular characterization of sickle cell anemia pediatric patients from two different cities in Brazil. Cad Saude Publica. 2005;21(4):1287–1290.
   PubMedGoogle Scholar
• Figueiredo MS, Kerbauy J, Goncalves MS, Effect of α-thalassemia and β-globin gene cluster haplotypes on the hematological and clinical features of sickle-cell anemia in Brazil. Am J Hematol. 1996;53(2):72–76.
   PubMed Web of Science ®Google Scholar
• el-Hazmi MA. Heterogeneity and variation of clinical and haematological expression of Haemoglobin S in Saudi Arabs. Acta Haematol. 1992;88(2–3):67–71.
   PubMedGoogle Scholar
• Embury SH, Dozy AM, Miller J, Davis JR Jr, WC. Concurrent sickle-cell anemia and α-thalassemia: effect on severity of anemia. N Engl J Med. 1982;306(5):270–274.
   PubMed Web of Science ®Google Scholar
• Stevens MC, Maude GH, Beckford M, α Thalassemia and the hematology of homozygous sickle cell disease in childhood. Blood. 1986;67(2):411–414.
   PubMed Web of Science ®Google Scholar
• de Ceulaer K, Higgs DR, Weatherall DJ, Hayes RJ, Serjeant BE, Serjeant GR. α-Thalassemia reduces the hemolytic rate in homozygous sickle-cell disease. N Engl J Med. 1983;309(3):189–190.
   PubMedGoogle Scholar
• Mukherjee MB, Colah RB, Ghosh K, Mohanty D, Krishnamoorthy R. Milder clinical course of sickle cell disease in patients with α thalassemia in the Indian subcontinent. Blood. 1997;89(2):732.
   PubMedGoogle Scholar
• Adorno EV, Zanette A, Lyra I, The β-globin gene cluster haplotypes in sickle cell anemia patients from Northeast Brazil: a clinical and molecular view. Hemoglobin. 2004;28(3):267–271.
   PubMed Web of Science ®Google Scholar
• Higgs DR, Vickers MA, Wilkie AOM, Pretorius I-M, Jarman AP, Weatherall DJ. A review of the molecular genetics of the human α-globin gene cluster. Blood. 1989;73(5):1081–1104.
   PubMed Web of Science ®Google Scholar
• Chang YC, Smith KD, Moore RD, Serjeant GR, Dover GJ. An analysis of fetal hemoglobin variation in sickle cell disease: the relative contributions of the X-linked factor, β-globin haplotypes, α-globin gene number, gender, and age. Blood. 1995;85(4):1111–1117.
   PubMed Web of Science ®Google Scholar
• De Ceulaer K, Serjeant GR. Acute splenic sequestration in Jamaican adults with homozygous sickle cell disease: a role of α thalassaemia. Br J Haematol. 1991;77(4):563–564.
   PubMedGoogle Scholar
• Castro O, Brambilla DJ, Thorington B, The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 1994;84(2):643–649.
   PubMed Web of Science ®Google Scholar
• Steinberg MH, Coleman MB, Adams JG, Rosenstock W. Interaction between Hb S-β0-thalassemia and α-thalassemia. Am J Med Sci. 1984;288(5):195–199.
   PubMedGoogle Scholar
• Vyas P, Higgs DR, Weatherall DJ, Dunn D, Serjeant BE, Serjeant GR. The interaction of α thalassaemia and sickle cell-β0 thalassaemia. Br J Haematol. 1988;70(4):449–454.
   PubMed Web of Science ®Google Scholar
• Gordis L, Ed. Cohort studies. Epidemiology, 2nd ed. Philadelphia: W.B. Saunders. 2000:131–139.
   Google Scholar
• Steinberg MH. Genetic etiologies for phenotypic diversity in sickle cell anemia. Scientific World Journal. 2009;9:46–67.
   Web of Science ®Google Scholar