Investigation of Lipid Profile and Clinical Manifestations in SCA Children

Abstract

Introduction: Clinical complications in sickle cell anemia (SCA) are heterogeneous and involve several molecules. It has been suggested that SCA individuals present a dyslipidemic phenotype and that lipid parameters are associated with severe clinical complications, such as pulmonary hypertension. We sought to investigate associations between lipid parameters and clinical manifestations, as well as other laboratory parameters in a population of pediatric SCA patients.

Methods: Our cross-sectional evaluation included 126 SCA patients in steady state and who were not undergoing lipid-lowering therapy. Hematological and biochemical parameters were characterized, and previous clinical manifestations were investigated.

Results: Total cholesterol and low-density lipoprotein cholesterol (LDL-C) levels were increased in patients with a previous history of pneumonia, which also positively correlated with HbS levels. Decreased LDL-C levels were also associated with leg ulcers and anemia. Elevated high-density lipoprotein cholesterol (HDL-C) levels were associated with pain crises, increased viscosity, and decreased hemolysis. Several studies have determined that lipids play a role in the vascular impairment seen in SCA, which was corroborated by our findings.

Conclusions: In sum, our results suggest that total cholesterol, HDL-C, and LDL-C levels are associated with hemolysis and anemia markers and, most importantly, with clinical complications related to vasculopathy in SCA.

Figure 1

Associations between total cholesterol, LDL-C, and HDL-C levels and clinical manifestations in SCA. (a) Patients with a previous history of pneumonia (N = 69) exhibited increased total cholesterol levels. (b) Patients with a previous history of leg ulcers (N = 12) presented decreased LDL-C levels. (c) Patients with a previous history of pain crises (N = 78) had increased HDL-C levels. p values obtained using the Mann-Whitney U test.

Figure 2

Correlations between lipid and hematological parameters in SCA patients with a previous history of pneumonia (N = 69) indicated by the brown circles. Patients without a previous history of pneumonia (N = 57) are indicated by the blue circles. (a) Total cholesterol levels were negatively correlated with mean platelet volume (MPV). (b) Total cholesterol levels were positively correlated with HbS levels. (c) LDL-C levels were positively correlated with HbS levels. (d) LDL-C levels were negatively correlated with MCV. (e) LDL-C levels were positively correlated with reticulocyte counts. (f) HDL-C levels were positively correlated with ferritin levels. Data comparisons made using Spearman's correlation rank test. None of the correlations described herein were statistically significant in patients without a previous history of pneumonia.

Figure 3

Correlations between HDL-C and triglyceride levels and hematological parameters in SCA patients with a previous history of pain crises (N = 78) indicated by the brown hexagons. Patients without a previous history of pain crises (N = 48) are indicated by the blue hexagons. (a) HDL-C levels were negatively correlated with RDW levels. (b) HDL-C levels were positively correlated with total cholesterol levels. (c) HDL-C levels were negatively correlated with HbF levels. (d) HDL-C levels were negatively correlated with MCHC. (e) Total cholesterol levels were positively correlated with albumin levels. (f) LDL-C levels were positively correlated with albumin levels. Data were compared using Spearman's correlation rank test. None of the correlations described herein were statistically significant in patients without a previous history of pain crises.