Profil biochimique et hématologique des patients drépanocytaires homozygotes en phase stationnaire au centre National de Transfusion Sanguine de Dakar

Doupa, Dominique; Djite, Moustapha; Gueye, Pape Madieye; Seck, Moussa; Faye, Blaise Felix; Seck, Sidy Mohamed; Diallo, Fatou; Ndiaye, A.; Samba, Abdourahmane; Cissé, Fatou; Diatta, Ampa Dogui; Diagne, Ibrahima; Diop, S.N.

doi:10.4314/ijbcs.v11i4.23

Cited by 7 publications

(13 citation statements)

References 12 publications

(15 reference statements)

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“…In our study, SS homozygotes had an average hemoglobin level of 8.1 g/dL. This mean level is close to those reported by Dahmani et al [11] and Doupa et al [12], with mean hemoglobin levels of 7.6 and 8.2 g/dL, respectively. The observed anemia was normocytic, with a mean corpuscular volume of 81.6 fL, which is in line with the literature.…”

Section: Discussionsupporting

confidence: 90%

Prevalence of Association of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease at the National Teaching Hospital of Cotonou in Benin

Zohoun,

Baglo-Agbodande,

Adje

et al. 2023

OJBD

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Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell disease are common genetic defects of red blood cells that lead to hemolytic anemia. The prevalence of G6PD deficiency in sickle cell patients is unknown in Benin. Objective: This study aimed to determine the prevalence of G6PD deficiency in sickle cell patients at the CNHU-HKM of Cotonou. Methods: This prospective study was conducted from April to November 2022 at the blood-related diseases teaching clinic and included sickle cell patients in the stationary phase. G6PD determination was performed using the enzymatic method on a Mindray BS 200 machine following the Herz method. Hematological parameters were determined using the XT 4000i analyzer and supplemented by a blood smear stained with May Grunwald Giemsa. Data were analyzed using Epi Info 3.5.4 software. Results: One hundred and sixty-four sickle cell patients (80 SS homozygotes and 84 SC heterozygotes) in the intercritical phase, with a mean age of 26.30 ± 10.76 years, were included. The prevalence of G6PD deficiency was 9.1% (15 cases found in 7 SS patients and 8 SC patients). In G6PD-deficient patients, the mean concentration of the enzyme was lower in Hb SC heterozygotes than in Hb SS homozygotes: 3.56 IU/g Hb versus 4.98 IU/g Hb. The mean reticulocyte count was 231.43 G/L in the deficient group, compared to 216.32 G/L in the non-deficient group. Conclusion: The preliminary results of our study reveal a high prevalence of G6PD deficiency in sickle cell patients. The impact of this association on he-

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Section: Discussionsupporting

confidence: 90%

Prevalence of Association of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease at the National Teaching Hospital of Cotonou in Benin

Zohoun,

Baglo-Agbodande,

Adje

et al. 2023

OJBD

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“…Indeed, an increase in CRP (> 6.00 mg/L) would be in favor of an inflammatory reaction. Our result was lower than that of the study by Doupa et al (2017) which reported a mean CRP value of 12.87 ± 21.9 mg/L (Doupa et al, 2017).…”

Section: Clinical and Biological Characteristics Of The Cohortcontrasting

confidence: 96%

Effects of Quantitative Trait Loci (QTLs) of BCL11A and HBS1L-MYB genes on clinical-biological variability of sickle cell disease in a Senegalese pediatric population

Tall¹,

Ndour²,

Djite³

et al. 2022

Afr. J. Biochem. Res.

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High levels of hemoglobin F (HbF) could reduce the severity of sickle cell disease (SCD) by inhibiting hemoglobin S (HbS) polymerization. HbF expression is modulated by the Quantitative Trait Loci (QTLs) located on the HBS1L-MYB intergenic region (HMIP) (rs28384513) and intron 2 of the B-cell lymphoma/leukemia 11A (BCL11A) gene (rs4671393, rs1427407). To assess the impact of QTLs of HbF on clinical and biological parameters associated with the severity of sickle cell disease in a Senegalese pediatric population, 301 children with SCD not treated with hydroxyurea were recruited. The numbers of hospitalizations and VOC were estimated over 2 years. HbF levels were determined by HPLC. Three QTLs of HbF were genotyped by High Resolution Melting (HRM), two single nucleotide polymorphisms (SNPs) of BCL11A and one SNP of HMIP. Data analysis was performed using SPSS. The mean frequency of hospitalizations was 0.84 ± 1.29. The mean number of vaso-occlusive crises (VOC) episodes was 2.73 ± 1.98. The mean Hb concentration was 7.76 ± 1.05 g/dl. The mean HbS was 82.28 ± 4.78% and the mean HbF was 9.49 ± 5.12%. BCL11A (rs1427407) was associated with fewer hospitalizations. Both BCL11A SNPs were associated with increased HbF levels. BCL11A SNPs were associated with increased HbF levels, decreased HbS levels, and decreased hospitalizations (rs1427407). However, no association was noted between these SNPs and the number of VOC episodes. Thus, HbF QTLs are not the only genetic factors modulating the clinical severity of sickle cell disease, which suggests the involvement of other genetic factors such as alpha-thalassemia.

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“…From the study, the sex ratio was 1.01 in favor of men which suggests equitable genetic transmission of the "S" allele regardless of sex. The following authors Dahmani et al (2016) and Doupa et al (2017) also reported equitable transmission of the condition between the two sexes. The study population was relatively young with a median age of 9 years (Al-Saqladi et al, 2012;Bandeira et al, 2014;Costacou et al, 2008;Da Guarda et al, 2020;Eric Kerchberger et al, 2019;Gudjoncik, 2018;Langlois & Delanghe, 1996;Santos et al, 2011;Soejima & Koda, 2020;Zahran et al, 2020).…”

Section: Discussionmentioning

confidence: 85%

“…(2016) and Doupa et al. (2017) also reported equitable transmission of the condition between the two sexes. The study population was relatively young with a median age of 9 years (Al‐Saqladi et al., 2012; Bandeira et al., 2014; Costacou et al., 2008; Da Guarda et al., 2020; Eric Kerchberger et al., 2019; Gudjoncik, 2018; Langlois & Delanghe, 1996; Santos et al., 2011; Soejima & Koda, 2020; Zahran et al., 2020).…”

Section: Discussionmentioning

confidence: 90%

“…Thrombocytosis in sickle cell patients is a factor promoting vaso‐occlusive crises because activated platelets secrete thrombospondin (TSP) involved in GR‐endothelium bypass and participate in the hypercoagulability state of sickle cell disease. It would also be the result of the occurrence of functional or organic asplenia or would be the consequence of hyposplenism (Doupa et al., 2017; Houwing et al., 2019). These previous descriptions made and reports suggest that in sickle cell disease, the “ HP 2 ” allele of haptoglobin would have a negative influence on the hematological profile.…”

Section: Discussionmentioning

confidence: 99%

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Haptoglobin gene polymorphism and iron profile in sickle cell disease patients with inflammation in Yaounde, Cameroon

Tuono,

Simo,

Nya

et al. 2023

Molec Gen & Gen Med

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BackgroundMajor sickle cell syndromes are the most common hemoglobinopathy in the world. The sickle cell patients are subjected to several factors causing inflammation, and the genetic identification of each individual allows to focus the possibility of allelic variations influence of a specific gene and then the polymorphism. This study aims at determining the distribution of HP gene (OMIM#140100) and their involvement on hematological parameters and the iron profile in the sickle cell patients presenting an inflammation condition during major sickle cell syndromes in Cameroun.MethodsA case–control analytical study has been conducted over a period of 6 months. Cases consisting of sickle cell patients in a situation of inflammation and control of non‐inflamed sickle cell patients. The patients presenting major sickle cell syndromes, interned and/or followed at the Hematology Department of the Regional Hospital of Bafoussam and the Central Hospital of Yaoundé have been recruited. HP genotyping was carried out at the Laboratory for Public Health Research Biotechnologies (LAPHER‐Biotech) in Yaoundé using allele‐specific PCR. Also, inflammatory, hematological parameters and martial assessment were explored by standard methods. Statistical analysis of the data was performed using the statistical tool R version 4.1.1. The comparison of proportions of alleles was made with the chi‐square test, and the Wilcoxon test was used to compare the median between different groups using the statistical tool R version 4.1.1.ResultsWe analyzed the samples of 149 patients. The HP polymorphism describes a significant frequency of the “1F” allele (69.8%) followed by the “2” allele (46.31%). In addition, 80 patients (53.69%), 48 (32.21%), and 21 (14.09%) presented the genotype HP 1‐1, HP 2‐1, and HP 2‐2, respectively. And eighty‐one percent (81%) patients with genotype HP 2‐2 showed a significant higher relative frequency of thrombocytosis compared with the genotype HP 1‐1 and HP 2‐1, respectively (51.2% and 68.8%, p = 0.087). The proportion of inflammation in the HP 2‐2 group was higher (57.1%) compared with the other groups (respectively 42.5% and 35.4% in the HP 1‐1 and HP 2‐1 groups). Furthermore, the median CRP was significantly higher in the HP 2‐2 group compared with the other groups (p = 0.039). Moreover, the entire population of the HP 2‐2 group showed an elevation of ferritin and IL6 unlike the HP 1‐1 and HP 2‐1 groups.ConclusionThis study demonstrates a higher frequency of genotype HP 1‐1 followed by the HP 2‐2 genotype in patients with major sickle cell syndromes. However, a larger proportion of patients with genotype HP 2‐2 are associated with hematological profile disorders, inflammation, and dysregulation of iron metabolism. Then, the haptoglobin polymorphism contributes to the severity of major sickle cell syndromes.

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Profil biochimique et hématologique des patients drépanocytaires homozygotes en phase stationnaire au centre National de Transfusion Sanguine de Dakar

Cited by 7 publications

References 12 publications

Prevalence of Association of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease at the National Teaching Hospital of Cotonou in Benin

Prevalence of Association of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease at the National Teaching Hospital of Cotonou in Benin

Effects of Quantitative Trait Loci (QTLs) of BCL11A and HBS1L-MYB genes on clinical-biological variability of sickle cell disease in a Senegalese pediatric population

Haptoglobin gene polymorphism and iron profile in sickle cell disease patients with inflammation in Yaounde, Cameroon

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