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

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 hematologic and biological parameters should be evaluated for better management of sickle cell disease.

S1P: the elixir of life for naive T cells

During their life,T cells are constantly circulating throughout the body.Sphingosine-1-phosphate(S1P),a sphingolipid metabolite,triggers T cell egress from the thymus and secondary lymphoid organs(SLO)into the lymph and the blood.This phenomenon is dependent on the S1P gradient between lymphoid organs(including thymus and SLO)and the lymphatic and blood vessels,which exhibit low,intermediate and high S1P levels,respectively.T cells follow the S1P gradient via the engagement of the G-protein-coupled receptor S1P receptor 1(S1P1)expressed on T cells.1 S1P production is regulated by various sphingolipid-metabolizing enzymes.The sphingosine kinases 1 and 2(SKs),encoded by Sphk1 and Sphk2,phosphorylate the sphingosine(Sph)into S1P.Conversely,S1P can be dephosphorylated to Sph by S1P phosphatases 1 and 2,encoded by Sgpp1 and Sgpp2.Alternatively,S1P can be irreversibly degraded by the S1P Lyase(SPL),encoded by Sgpl1,into phosphoethanolamine and hexadecenal.