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Blood, 15 November 2008, Vol. 112, No. 10, pp. 4314-4317.
Prepublished online as a Blood First Edition Paper on September 4, 2008; DOI 10.1182/blood-2008-03-143891.


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RED CELLS

Brief Report

G6PD deficiency, absence of {alpha}-thalassemia, and hemolytic rate at baseline are significant independent risk factors for abnormally high cerebral velocities in patients with sickle cell anemia

Françoise Bernaudin1, Suzanne Verlhac2, Sylvie Chevret3, Martine Torres4, Lena Coic1, Cécile Arnaud1, Annie Kamdem1, Isabelle Hau1, Maria Grazia Neonato5, and Christophe Delacourt1

1 Reference Center for Sickle Cell Disease, Department of Pediatrics, and 2 Medical Imagerie, Department of Radiology, Intercommunal Créteil Hospital, Créteil, France; 3 Department of Statistics, St-Louis Hospital, Paris, France; 4 Pediatrics, University of Southern California, Los Angeles; and 5 Hematology, Molecular Biology, Tenon Hospital, Paris, France

Stroke is predicted by abnormally high cerebral velocities by transcranial doppler (TCD). This study aimed at defining predictive factors for abnormally high velocities (≥ 2 m/sec) based on the Créteil pediatric sickle cell anemia (SCA) cohort composed of 373 stroke-free SCA children. {alpha} genes and β-globin haplotypes were determined. Biologic parameters were obtained at baseline. {alpha}-thalassemia was present in 155 of 325 and G6PD deficiency in 36 of 325 evaluated patients. TCD was abnormal in 62 of 373 patients. Multivariate logistic regression analysis showed that G6PD deficiency (odds ratio [OR] = 3.36, 95% confidence interval [CI] 1.10-10.33; P = .034), absence of alpha-thalassemia (OR = 6.45, 95% CI 2.21-18.87; P = .001), hemoglobin (OR per g/dL = 0.63, 95% CI 0.41-0.97; P = .038), and lactate dehydrogenase (LDH) levels (OR per IU/L = 1.001, 95% CI 1.000-1.002; P = .047) were independent risk factors for abnormally high velocities. This study confirms the protective effect of alpha-thalassemia and shows for the first time that G6PD deficiency and hemolysis independently increase the risk of cerebral vasculopathy.


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Related Article in Blood Online:

Glucose 6 phosphate dehydrogenase deficiency is not associated with cerebrovascular disease in children with sickle cell anemia
David C. Rees, Christopher Lambert, Elaine Cooper, Jack Bartram, David Goss, Colin Deane, and Swee Lay Thein
Blood 2009 114: 742-743. [Full Text] [PDF]



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D. C. Rees, C. Lambert, E. Cooper, J. Bartram, D. Goss, C. Deane, and S. L. Thein
Glucose 6 phosphate dehydrogenase deficiency is not associated with cerebrovascular disease in children with sickle cell anemia
Blood, July 16, 2009; 114(3): 742 - 743.
[Full Text] [PDF]



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