title: Association of classical markers and establishment of the dyslipidemic sub-phenotype of sickle cell anemia.
abstract: BACKGROUND
  Sickle cell anemia (SCA) patients exhibit sub-phenotypes associated to hemolysis and vaso-occlusion. The disease has a chronic inflammatory nature that has been also associated to alterations in the lipid profile. This study aims to analyze hematological and biochemical parameters to provide knowledge about the SCA sub-phenotypes previously described and suggest a dyslipidemic sub-phenotype.
  
  
  METHODS
  A cross-sectional study was conducted from 2013 to 2014, and 99 SCA patients in steady state were enrolled. We assessed correlations and associations with hematological and biochemical data and investigated the co-inheritance of -α3.7Kb-thalassemia (-α3.7Kb-thal). Correlation analyses were performed using Spearman and Pearson coefficient. The median of quantitative variables between two groups was compared using t-test and Mann-Whitney. P-values <0.05 were considered statistically significant.
  
  
  RESULTS
  We found significant association of high lactate dehydrogenase levels with decreased red blood cell count and hematocrit as well as high levels of total and indirect bilirubin. SCA patients with low nitric oxide metabolites had high total cholesterol, high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol and reduced very low-density cholesterol, triglycerides, direct bilirubin level and reticulocyte counts. In SCA patients with high-density lipoprotein cholesterol greater than 40 mg/dL, we observed increased red blood cell count, hemoglobin, hematocrit, and fetal hemoglobin and decreased nitric oxide metabolites levels. The presence of -α3.7Kb-thal was associated with high red blood cell count and low mean corpuscular volume, mean corpuscular hemoglobin, platelet count and total and indirect bilirubin levels.
  
  
  CONCLUSIONS
  Our results provide additional information about the association between biomarkers and co-inheritance of -α3.7Kb-thal in SCA, and suggest the role of dyslipidemia and nitric oxide metabolites in the characterization of this sub-phenotype.
authors.affiliation: Laboratório de Hematologia e Genética Computacional, Instituto Gonçalo Moniz - IGM, Rua Waldemar Falcão, 121, Candeal - Salvador/BA, CEP: 40296-710, Bahia, Brazil.|Universidade Federal da Bahia (UFBA), Salvador, Bahia, Brazil.
authors.forename: Milena Magalhães
authors.initials: MM
authors.lastname: Aleluia
authors.affiliation: Laboratório de Hematologia e Genética Computacional, Instituto Gonçalo Moniz - IGM, Rua Waldemar Falcão, 121, Candeal - Salvador/BA, CEP: 40296-710, Bahia, Brazil.|Universidade Federal da Bahia (UFBA), Salvador, Bahia, Brazil.
authors.forename: Caroline Conceição
authors.initials: CC
authors.lastname: da Guarda
authors.affiliation: Laboratório de Hematologia e Genética Computacional, Instituto Gonçalo Moniz - IGM, Rua Waldemar Falcão, 121, Candeal - Salvador/BA, CEP: 40296-710, Bahia, Brazil.|Universidade Federal da Bahia (UFBA), Salvador, Bahia, Brazil.
authors.forename: Rayra Pereira
authors.initials: RP
authors.lastname: Santiago
authors.affiliation: Centro de Referência a Doença Falciforme de Itabuna, Itabuna, Bahia, Brazil.|Universidade Estadual de Santa Cruz (UESC), Ilhéus, Bahia, Brazil.
authors.forename: Teresa Cristina Cardoso
authors.initials: TC
authors.lastname: Fonseca
authors.affiliation: Centro de Referência a Doença Falciforme de Itabuna, Itabuna, Bahia, Brazil.
authors.forename: Fábia Idalina
authors.initials: FI
authors.lastname: Neves
authors.affiliation: Centro de Referência a Doença Falciforme de Itabuna, Itabuna, Bahia, Brazil.|Universidade Estadual de Santa Cruz (UESC), Ilhéus, Bahia, Brazil.
authors.forename: Regiana Quinto
authors.initials: RQ
authors.lastname: de Souza
authors.affiliation: Universidade Estadual de Santa Cruz (UESC), Ilhéus, Bahia, Brazil.
authors.forename: Larissa Alves
authors.initials: LA
authors.lastname: Farias
authors.affiliation: Universidade Estadual de Santa Cruz (UESC), Ilhéus, Bahia, Brazil.
authors.forename: Felipe Araújo
authors.initials: FA
authors.lastname: Pimenta
authors.affiliation: Laboratório de Hematologia e Genética Computacional, Instituto Gonçalo Moniz - IGM, Rua Waldemar Falcão, 121, Candeal - Salvador/BA, CEP: 40296-710, Bahia, Brazil.|Universidade Federal da Bahia (UFBA), Salvador, Bahia, Brazil.
authors.forename: Luciana Magalhães
authors.initials: LM
authors.lastname: Fiuza
authors.affiliation: Laboratório de Hematologia e Genética Computacional, Instituto Gonçalo Moniz - IGM, Rua Waldemar Falcão, 121, Candeal - Salvador/BA, CEP: 40296-710, Bahia, Brazil.
authors.forename: Thassila Nogueira
authors.initials: TN
authors.lastname: Pitanga
authors.affiliation: Laboratório de Hematologia e Genética Computacional, Instituto Gonçalo Moniz - IGM, Rua Waldemar Falcão, 121, Candeal - Salvador/BA, CEP: 40296-710, Bahia, Brazil.|Universidade Federal da Bahia (UFBA), Salvador, Bahia, Brazil.
authors.forename: Júnia Raquel Dutra
authors.initials: JR
authors.lastname: Ferreira
authors.affiliation: Universidade Federal da Bahia (UFBA), Salvador, Bahia, Brazil.
authors.forename: Elisângela Vitória
authors.initials: EV
authors.lastname: Adorno
authors.affiliation: Universidade Estadual da Bahia (UNEB), Salvador, Bahia, Brazil.
authors.forename: Bruno Antônio Veloso
authors.initials: BA
authors.lastname: Cerqueira
authors.affiliation: Laboratório de Hematologia e Genética Computacional, Instituto Gonçalo Moniz - IGM, Rua Waldemar Falcão, 121, Candeal - Salvador/BA, CEP: 40296-710, Bahia, Brazil. mari@bahia.fiocruz.br.|Universidade Federal da Bahia (UFBA), Salvador, Bahia, Brazil. mari@bahia.fiocruz.br.
authors.forename: Marilda de Souza
authors.initials: MS
authors.lastname: Gonçalves
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications / Q000235:genetics / Q000503:physiopathology*; D001663:Bilirubin / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001938:Brazil; D003430:Cross-Sectional Studies; D050171:Dyslipidemias / Q000209:etiology*; D004906:Erythrocyte Count; D004909:Erythrocyte Indices; D017353:Gene Deletion; D006400:Hematocrit; D006447:Hemoglobin H / Q000235:genetics; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008055:Lipids / Q000097:blood; D009569:Nitric Oxide / Q000097:blood; D010976:Platelet Count; D017085:alpha-Thalassemia / Q000150:complications / Q000235:genetics
chemical_list: D015415:Biomarkers; D008055:Lipids; D009569:Nitric Oxide; D006447:Hemoglobin H; D007770:L-Lactate Dehydrogenase; D001663:Bilirubin
keywords: Dyslipidemia; Sickle cell anemia; Sub-phenotype; α-thalassemia