Abstract
BackgroundHypocholesterolemia is the most frequently encountered lipid abnormality in sickle cell disease (SCD). We enrolled pediatric patients to determine the relationships between lipid profile and parameters of hemolysis, oxidative stress and chronic inflammation in SCD.MethodsThe study involved 35 pediatric SCD patients and 19 healthy controls. Patients were crisis-free and had not received transfusions for the last 3 months. Total cholesterol, triglyceride, HDL-C, LDL-C, VLDL-C, apolipoprotein A1, apolipoprotein B, LCAT, LDH, bilirubin, haptoglobin, iron, ferritin, hemin, serum amyloid A (SAA), myeloperoxidase (MPO), uric acid, ALT and GGT levels were evaluated in patients’ blood.ResultsPatients had hypocholesterolemia depicted by lower levels of total cholesterol, HDL-C, LDL-C, as well as Apolipoprotein A1 and Apolipoprotein B compared to controls. The chronic hemolysis of SCD was evident in patients by higher LDH and bilirubin and almost undetectable haptoglobin levels. Hemin levels (as a measure of oxidized heme) were significantly increased in patients with SCD. Inflammation markers, SAA and MPO, were significantly increased in the patients as well. There were negative correlations between HDL-C and LDH, and Apo A1 and SAA. Hemin was positively correlated to MPO.ConclusionHemolysis was associated with decreased HDL –C, and Inflammation was linked to decreased apolipoprotein A1 levels in our SCD patients. Therefore, we suggest that the HDL particle is altered during the course of the disease. The altered HDL in SCD may become dysfunctional and result with a slowing down of the reverse cholesterol transport.
Highlights
Sickle cell disease (SCD) is an inherited hemoglobinopathy, caused by a point mutation in the beta globin gene that induces abnormal hemoglobin production (Hemoglobin S, Hemoglobin sickle (HbS)) [1]
The major findings of the study were the association between hemolysis and decreased High density lipoprotein (HDL)-C, as well as the link between inflammation and decreased apolipoprotein A1 levels
Hemin levels were found to be correlated positively with MPO levels, suggesting that hemin may play an important role in the activation of inflammation in patients with sickle cell disease (SCD), which may in turn aggravate the oxidative damage sustained by erythrocytes, endothelial cells, HDL and Low density lipoprotein (LDL). These results suggest that hemin levels may contribute to inflammatory activation and possibly the oxidation of the apolipoprotein A1 (Apo A1) of the HDL particle in SCD patients; causing HDL dysfunction
Summary
Sickle cell disease (SCD) is an inherited hemoglobinopathy, caused by a point mutation in the beta globin gene that induces abnormal hemoglobin production (Hemoglobin S, HbS) [1]. Sickle erythrocytes become prone to SCD is a chronic condition and patients have increased hemolytic, oxidant and inflammatory stress, even at the steady state of the disease [4]. Anemia is a result of chronic hemolysis, induced by continuous sickling of Yalcinkaya et al Lipids in Health and Disease (2019) 18:225 the erythrocyte. This may induce Hb denaturation and lead to increased oxidative stress through various mechanisms. Of these mechanisms, higher methemoglobin levels, heme and iron release into the circulation, and hemin formation from free heme are the most prominent. We enrolled pediatric patients to determine the relationships between lipid profile and parameters of hemolysis, oxidative stress and chronic inflammation in SCD
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