Abstract

BackgroundSickle 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.MethodsA 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 -α3.7Kb-thalassemia (-α3.7Kb-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.ResultsWe 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 -α3.7Kb-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.ConclusionsOur results provide additional information about the association between biomarkers and co-inheritance of -α3.7Kb-thal in SCA, and suggest the role of dyslipidemia and nitric oxide metabolites in the characterization of this sub-phenotype.

Highlights

  • Sickle cell anemia (SCA) patients exhibit sub-phenotypes associated to hemolysis and vaso-occlusion

  • The vasoocclusive sub-phenotype has been associated with blood viscosity and vaso-occlusive episodes (VOE), and the hemolytic sub-phenotype has been related with hemolysis and endothelial dysfunction, with alterations in nitric oxide (NO) and lactate dehydrogenase (LDH) levels, as well as hematological parameters [5]

  • 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 to SCA

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Summary

Introduction

Sickle cell anemia (SCA) patients exhibit sub-phenotypes associated to hemolysis and vaso-occlusion. SCA is characterized by hemolysis, chronic and acute inflammation, vaso-occlusive complications, multiple organ damage, and reduced patient survival [1]. The changes in the blood flow may contribute to increase oxidative stress, leading to vaso-occlusive episodes (VOE) complications, such as acute chest syndrome (ACS), stroke, priapism, gallstone, and retinopathy [3, 4]. SCA patients exhibit sub-phenotypes that very often overlap, they are helpful to understand the pathophysiological mechanisms of the disease. The vasoocclusive sub-phenotype has been associated with blood viscosity and VOE, and the hemolytic sub-phenotype has been related with hemolysis and endothelial dysfunction, with alterations in nitric oxide (NO) and lactate dehydrogenase (LDH) levels, as well as hematological parameters [5]

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