Association of classical markers and establishment of the dyslipidemic sub-phenotype of sickle cell anemia

Aleluia, Milena Magalhães; Guarda, Caroline Conceição da; Santiago, Rayra Pereira; Fonseca, Tamara Canto; Neves, Fábia Idalina; Souza, Regiana Quinto de; Farias, Larissa Alves; Pimenta, Felipe Araújo; Fiuza, Luciana Magalhães; Pitanga, Thassila Nogueira; Ferreira, Júnia Raquel Dutra; Adôrno, Elisângela Vitória; Cerqueira, Bruno A. V.; Gonçalves, Marilda de Souza

doi:10.1186/s12944-017-0454-1

Cited by 22 publications

(26 citation statements)

References 27 publications

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“…Thus, the association of both pathophysiological mechanisms to the triad of factors (VO, inflammation and nociception) may help to initiate the acute painful crises [37]. Abnormal lipid homeostasis has also been associated with decreased NOm levels [6].…”

Section: Discussionmentioning

confidence: 99%

“…It is thought that PH, leg ulcer and stroke are associated to the chronic hemolytic feature of SCD, while acute pain crises, osteonecrosis and ACS are associated to VO, which could drive to different subphenotypes [5]. However, this dichotomization is not restricted, very often they overlap and may not be useful for SCA and HbSC individually [5][6][7]. Moreover, recently it has been suggested that abnormal lipid homeostasis would be surrogate subphenotype, considering the association with both hemolysis and VO [6].…”

Section: Introductionmentioning

confidence: 99%

“…However, this dichotomization is not restricted, very often they overlap and may not be useful for SCA and HbSC individually [5][6][7]. Moreover, recently it has been suggested that abnormal lipid homeostasis would be surrogate subphenotype, considering the association with both hemolysis and VO [6]. SCA patients usually present clinical events more frequently when compared to HbSC disease, which is considered a milder form of SCD [1,4,8].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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Sickle cell disease (SCD) consists of a group of hemoglobinopathies in which individuals present highly variable clinical manifestations. Sickle cell anemia (SCA) is the most severe form, while SC hemoglobinopathy (HbSC) is thought to be milder. Thus, we investigated the clinical manifestations and laboratory parameters by comparing each SCD genotype. We designed a cross-sectional study including 126 SCA individuals and 55 HbSC individuals in steady-state. Hematological, biochemical and inflammatory characterization was performed as well as investigation of previous history of clinical events. SCA patients exhibited most prominent anemia, hemolysis, leukocytosis and inflammation, whereas HbSC patients had increased lipid determinations. The main cause of hospitalization was pain crises on both genotypes. Vaso-occlusive events and pain crises were associated with hematological, inflammatory and anemia biomarkers on both groups. Cluster analysis reveals hematological, inflammatory, hemolytic, endothelial dysfunction and anemia biomarkers in HbSC disease as well as SCA. The results found herein corroborate with previous studies suggesting that SCA and HbSC, although may be similar from the genetic point of view, exhibit different clinical manifestations and laboratory alterations which are useful to monitor the clinical course of each genotype.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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“…The present results demonstrate the beneficial effect of HU on cholesterol, since it is known that plasma lipids, particularly high levels of triglycerides, are correlated with markers of intravascular hemolysis, vascular dysfunction, and pulmonary hypertension in sickle cell disease patients [ 35 ]. Recently, our research group proposed a dyslipidemic subphenotype in SCA patients [ 36 ]. Significant decreases in LDH, TB, DB, and IB were seen among SCA-HU + patients, suggesting an improvement in the hemolytic profile.…”

Section: Discussionmentioning

confidence: 99%

Sickle Cell Anemia Patients in Use of Hydroxyurea: Association between Polymorphisms in Genes Encoding Metabolizing Drug Enzymes and Laboratory Parameters

et al. 2018

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This study investigated associations between SNPs in genes encoding metabolizing drug enzymes and laboratory parameters in sickle cell anemia patients under hydroxyurea (SCA-HU+). We evaluated hematologic and biochemical parameters by electronic methods and SNPs by PCR-RFLP and multiplex PCR in 35 SCA-HU+ patients and 67 SCA-HU− patients. The HbS, total cholesterol, lactate dehydrogenase, aspartate aminotransferase, total bilirubin and fractions levels, and leukocyte, eosinophil, monocyte, and erythroblast counts were reduced in SCA-HU+ patients (p < 0.05). Moreover, they presented higher HbF, C-reactive protein, and ferritin levels and elevated MCH and MCV values (p < 0.05). Genotype frequencies of variants GA + AA of MPO −463G>A and c1c2 + c2c2 of CYP2E1 −1293G>C/−1053C>T were higher in SCA-HU+ patients (p < 0.05). Independent associations were found between the variant A allele and lower total cholesterol, between c2 allele and low alpha-1 antitrypsin and between the null GSTT1 variant and high indirect and total bilirubin in SCA-HU+ patients. In SCA-HU− patients, independent associations were found between the variant A allele and high uric acid and between c2 allele and high urea. Our results suggest that SNPs MPO −463G>A, CYP2E1 −1293G>C/−1053C>T, and GSTT1 can be associated with alterations in lipid, inflammatory, renal, hemolytic, and hepatic profiles. However, further studies are needed to elucidate these associations.

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“…Contrasting results have been reported regarding the level of triglycerides (TG) in SCA compared to controls [ 7 , 9 , 10 ]. This dyslipidemia has been associated with the severity of hemolysis and would be involved in vascular dysfunction [ 7 , 11 , 12 ]. In addition, it has been also shown that patients with the highest TG levels would be prone to develop complications like pulmonary hypertension [ 7 , 11 , 12 ] and acute chest syndrome [ 13 ].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2018

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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.

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Association of classical markers and establishment of the dyslipidemic sub-phenotype of sickle cell anemia

Cited by 22 publications

References 27 publications

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

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

Sickle Cell Anemia Patients in Use of Hydroxyurea: Association between Polymorphisms in Genes Encoding Metabolizing Drug Enzymes and Laboratory Parameters

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

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