Lipid profiles in French West Indies sickle cell disease cohorts, and their general population

Abstract

BackgroundThe pathophysiology of sickle cell disease (SCD) and the variability of its clinical expression remain not fully understood, whether within or between different SCD genotypes. Recent studies have reported associations between lipid levels and several SCD complications. If lipid levels have been previously described as low in sickle cell anemia (SCA), few data have been provided for sickle cell SC disease (SCC). We designed our epidemiological study to isolate lipid levels and profiles by genotype in Guadeloupian cohorts of SCA and SCC adult patients, at steady state. We compared SCD lipid levels with those of the Guadeloupian general population (GGP), and analyzed potential associations between lipid levels and SCD complications (vaso-occlusive crises, acute chest syndrome and osteonecrosis).MethodsLipids, apolipoproteins, biological variables and anthropometric evaluation, were collected at steady state from medical files for 62 SCC and 97 SCA adult patients. Clinical SCD complications were collected from the clinical files. Analysis was conducted by genotype for all variables.ResultsDifferent SCC and SCA lipid profiles, both distinct from their GGP’s, were identified. Compared to SCC and GGP, higher triglyceride (TG) levels were observed in SCA patients, independent of hydroxyurea, hemolysis, gender, age, body mass index (BMI), abdominal obesity and clinical nutritional status. Our survey highlights also subsequent anthropometrical phenotypes, with an over-representation of abdominal obesity with normal BMI in SCA patients, and affecting almost exclusively females in both genotypes. Moreover, more frequent positive history of acute chest syndrome (ACS) was observed in SCA patients with TG level higher than 1.50 g/l, and of osteonecrosis in SCC patients having non high-density lipoprotein-cholesterol level (Non HDL-C) higher than 1.30 g/l.ConclusionsThis study reveals that SCA and SCC patients exhibit distinct lipid profiles and suggests that high TG and Non HDL-C levels are associated with past histories of ACS and osteonecrosis in SCA and SCC patients, respectively.

Sickle cell disease: A distinction of two most frequent genotypes (HbSS and HbSC)

“…Our findings are in agreement with previous laboratory profile of HbSC disease [26], including increased creatinine levels [27] and increased total cholesterol, HDL-C and LDL-C as well as decreased NOm determinations [8]. This lipid profile among HbSC individuals has also been show in other populations [28].…”

Type 2 diabetes in adults with sickle cell disease: can we dive deeper?

“…Lipid profile and anthropometrics, two factors that are associated with T2D pathogenesis, also differ between SCA and HbSC disease. A recent study (Lalanne‐Mistrih et al , ) showed that SCA and HbSC subjects had distinct lipid profiles that also differed from the general population. Notably, SCA subjects had higher triglyceride levels, which were associated with ACS, while non‐high density lipoprotein‐cholesterol levels were associated with osteonecrosis in HbSC subjects (Lalanne‐Mistrih et al , ).…”

“…A recent study (Lalanne‐Mistrih et al , ) showed that SCA and HbSC subjects had distinct lipid profiles that also differed from the general population. Notably, SCA subjects had higher triglyceride levels, which were associated with ACS, while non‐high density lipoprotein‐cholesterol levels were associated with osteonecrosis in HbSC subjects (Lalanne‐Mistrih et al , ). Furthermore, obesity has been shown to be on the rise in people with SCD, particularly in those with HbSC (Ballas, ).…”

“…Furthermore, obesity has been shown to be on the rise in people with SCD, particularly in those with HbSC (Ballas, ). Interestingly, the results of Lalanne‐Mistrih et al () revealed that mean body mass index (BMI) was significantly lower in the SCA compared to the HbSC subjects. However, there was an over‐representation of individuals with a normal BMI combined with abdominal obesity in the SCA group (Lalanne‐Mistrih et al , ).…”

“…Interestingly, the results of Lalanne‐Mistrih et al () revealed that mean body mass index (BMI) was significantly lower in the SCA compared to the HbSC subjects. However, there was an over‐representation of individuals with a normal BMI combined with abdominal obesity in the SCA group (Lalanne‐Mistrih et al , ). Current estimates indicate that 60% of the SCD population in the US has SCA, and 30% has HbSC disease (Hassell, ).…”