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Association of Classical Markers and Establishment of the Dyslipidemic Sub-phenotype of Sickle Cell Anemia

Abstract

Background: Sickle cell anemia (SCA) patients exhibit sub-phenotypes associated to hemolysis and vaso-occlusion. The disease has a chronic inflammatory nature that has been also associated to alterations in the lipid profile. This study aims to analyze hematological and biochemical parameters to provide knowledge about the SCA sub-phenotypes previously described and suggest a dyslipidemic sub-phenotype.

Methods: A cross-sectional study was conducted from 2013 to 2014, and 99 SCA patients in steady state were enrolled. We assessed correlations and associations with hematological and biochemical data and investigated the co-inheritance of -α-thalassemia (-α-thal). Correlation analyses were performed using Spearman and Pearson coefficient. The median of quantitative variables between two groups was compared using t-test and Mann-Whitney. P-values <0.05 were considered statistically significant.

Results: We found significant association of high lactate dehydrogenase levels with decreased red blood cell count and hematocrit as well as high levels of total and indirect bilirubin. SCA patients with low nitric oxide metabolites had high total cholesterol, high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol and reduced very low-density cholesterol, triglycerides, direct bilirubin level and reticulocyte counts. In SCA patients with high-density lipoprotein cholesterol greater than 40 mg/dL, we observed increased red blood cell count, hemoglobin, hematocrit, and fetal hemoglobin and decreased nitric oxide metabolites levels. The presence of -α-thal was associated with high red blood cell count and low mean corpuscular volume, mean corpuscular hemoglobin, platelet count and total and indirect bilirubin levels.

Conclusions: Our results provide additional information about the association between biomarkers and co-inheritance of -α-thal in SCA, and suggest the role of dyslipidemia and nitric oxide metabolites in the characterization of this sub-phenotype.

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References
1.
Schaer D, Buehler P, Alayash A, Belcher J, Vercellotti G . Hemolysis and free hemoglobin revisited: exploring hemoglobin and hemin scavengers as a novel class of therapeutic proteins. Blood. 2012; 121(8):1276-84. PMC: 3578950. DOI: 10.1182/blood-2012-11-451229. View

2.
Kato G, Gladwin M, Steinberg M . Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes. Blood Rev. 2006; 21(1):37-47. PMC: 2048670. DOI: 10.1016/j.blre.2006.07.001. View

3.
Rumaney M, Ngo Bitoungui V, Vorster A, Ramesar R, Kengne A, Ngogang J . The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival. PLoS One. 2014; 9(6):e100516. PMC: 4076272. DOI: 10.1371/journal.pone.0100516. View

4.
Seixas M, Rocha L, Carvalho M, Menezes J, Lyra I, Nascimento V . Levels of high-density lipoprotein cholesterol (HDL-C) among children with steady-state sickle cell disease. Lipids Health Dis. 2010; 9:91. PMC: 2940866. DOI: 10.1186/1476-511X-9-91. View

5.
Adegoke S, Adeodu O, Adekile A . Sickle cell disease clinical phenotypes in children from South-Western, Nigeria. Niger J Clin Pract. 2014; 18(1):95-101. DOI: 10.4103/1119-3077.146987. View