Elevated Levels of Hepatokine Angiopoietin-like 3 Correlate Paradoxically with Hypocholesterolemia and Hemolysis in Sickle Cell Anemia

Sickle cell disease (SCD) is characterized by chronic hemolytic anemia and vaso-occlusion. Hypocholesterolemia has been long recognized in SCD patients, and while it has been postulated that it is secondary to increased erythropoiesis, these mechanisms are not fully understood. Despite their name, angiopoietin-like proteins (ANGPTLs) are family of regulators of angiogenesis unable to bind to receptors classically targeted by angiopoetins. ANGPTL3 and ANGPTL4 are also inhibitors of lipoprotein lipase (LPL), and have become pharmacological targets in the treatment of dyslipidemia. We hypothesized that ANGPTL levels would be abnormal in SCD and could contribute to the understanding of lipid dysregulation. We aimed to determine, compare and correlate the circulating concentrations of ANGPTL3 and ANGPTL4 with hemolysis, lipid metabolism and endothelial dysfunction biomarkers in patients with sickle cell anemia (HbSS), hemoglobin SC disease (HbSC), and healthy individuals (HbAA). Thirty-six HbSS patients (age range 18-55, 21 men), 19 HbSC patients (HbSC) (age range 29-68, 8 men), and 31 HbAA controls (age range 18-66, 23 men) were enrolled in this study. Exclusion criteria were pregnancy, history of blood transfusion or sickle cell pain crisis in the past 3 months. Peripheral blood samples were collected for complete blood counts, biochemistry tests (lactate dehydrogenase [LDH], bilirubin, cholesterol and low and high density fractions, oxidized LDL [oxLDL], hemopexin), and determi...
Source: Blood - Category: Hematology Authors: Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster I Source Type: research