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.
References
[1]
Luzzatto, L. and Seneca, E. (2014) G6PD Deficiency: A Classic Example of Pharmacogenetics with On-Going Clinical Implications. British Journal of Haematology, 164, 469-480. https://doi.org/10.1111/bjh.12665
[2]
Wajcman, H. and Galacteros, F. (2004) Glucose 6-Phosphate Dehydrogenase Deficiency: A Protection against Malaria and a Risk for Hemolytic Accidents. Comptes Rendus Biologies, 327, 711-720. https://doi.org/10.1016/j.crvi.2004.07.010
[3]
Fasola, F.A., Fowodu, F.O., Shokunbi, W.A. and Kotila, T.R. (2019) The Effect of the Coinheritance of Glucose-6-Phosphate Dehydrogenase Deficiency on the Severity of Sickle Cell Disease. Nigerian Postgraduate Medical Journal, 26, 118-122. https://doi.org/10.4103/npmj.npmj_29_19
[4]
Antwi-Baffour, S., Adjei, K., Forson, P., Akakpo, S., Kyeremeh, R. and Seidu, M. (2019) Comorbidity of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease Exert Significant Effect on RBC Indices. Anemia, 2019, Article ID: 3179173. https://doi.org/10.1155/2019/3179173
[5]
Shivwanshi, R., Singh, E. and Kumar, A. (2019) A Positive Correlation between Sickle Cell Anemia and G6PD Deficiency from Population of Chhattisgarh, India. Gene, 707, 143-150. https://doi.org/10.1016/j.gene.2019.04.080
[6]
Herz, F., Kaplan, E. and Scheye, E.S. (1970) Diagnosis of Erythrocyte G6PD Deficiency in the Negro Male Despite Hemolytic Crisis. Blood, 35, 90-93. https://doi.org/10.1182/blood.V35.1.90.90
[7]
Latoundji, S., Gangni, I. and Zohoun, I. (1991) L’hémogramme chez le béninois adulte. Afrique Médicale, 38, 8-9.
[8]
WHO Working Group (1989) Glucose-6-Phosphate Dehydrogenase Deficiency. Bulletin World Health Organization, 67, 601-611. https://apps.who.int/iris/bitstream/handle/10665/264721/PMC2491315.pdf
[9]
Bouanga, J., Mouélé, R., Préhu, C., Wajcman, H., Feingold, J. and Galactéros, F. (1998) Glucose-6-Phosphate Dehydrogenase Deficiency and Homozygous Sickle Cell Disease in Congo. Human Heredity, 48, 192-197. https://doi.org/10.1159/000022801
[10]
Diop, S., Thiam, D., Sene, A., Cisse, M., Fall, K., Diakhate, L., et al. (2000) Association Drepanocytose-déficit en G6PD: Prévalence et influence sur le profil évolutif. Médecine d’Afrique Noire, 47, 322-326. http://www.santetropicale.com/Resume/74703.pdf
[11]
Dahmani, F., Benkirane, S., Kouzih, J., Woumki, A., Mamad, H. and Masrar, A. (2016) Etude de l’hémogramme dans la drépan ocytose homozygote: à propos de 87 patients. The Pan African Medical Journal, 25, Article 240. https://doi.org/10.11604/pamj.2016.25.240.11118
[12]
Doupa, D., Djite, M., Gueye, P., Seck, M., Faye, B., Diallo, F., et al. (2017) Profil biochimique et hématologique des patients drépanocytaires homozygotes en phase stationnaire au Centre National de Transfusion Sanguine de Dakar. International Journal of Biological and Chemical Sciences, 11, 1706-1715. https://doi.org/10.4314/ijbcs.v11i4.23
[13]
Nacoulma, E., Sakande, J., Kafando, E., Kpowbié, D. and Guissou, P. (2006) Profil hematologique et biochimique des drepanocytaires SS et SC en phase stationnaire au Centre Hospitalier National Yalgado Ouedraogo de Ouagadougou. Mali Médical, 21, 8-11.
[14]
Marie-Redelsperger, M., Bardakdjlan-Michau, J., Neonato, M.G. and Girot, R. (2003) Diagnostic biologique des syndromes drépanocytaires. In: Girot, R., Bégué, P. and Galacteros, F., Eds., La drépanocytose, John Libbey Eurotext, Paris, 13-29.
[15]
Wajcman, H. (2006) Déficits en glucose-6-phosphate-déshydrogénase. EMC (Elsevier SAS, Paris), Hématologie, 13-006-D-10.
[16]
Varret, M., Gudrid, L., Bikoue, A., Brunel, O. and Etienne, N. (2020) Study of G6PD Deficit (Glucose-6-Phosphate Deshydrogenase) in Blood Donors in Brazzaville. Biomedical Sciences, 6, 84-88. https://doi.org/10.11648/j.bs.20200604.12
[17]
Boutchi, M., Ibrahim, A., Daouda, A., Zeinabou, S. and Touré, A. (2016) Sickle Cell Anemia and Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency: Impact on Biological and Clinical Parameters. International Journal of Biotech Trends and Technology, 6, 27-31.
[18]
Saad, S. and Costa, F. (1992) Glucose-6-Phostate Deficiency and Sickle Cell Disease in Brazil. Human Heredity, 42, 125-128. https://doi.org/10.1159/000154052
[19]
Steinberg, M.H., West, M.S., Gallagher, D. and Mentzer, W. (1998) Effects of Glucose-6-Phosphate Dehydrogenase Deficiency upon Sickle Cell Anemia. Blood, 71, 748-752. https://doi.org/10.1182/blood.V71.3.748.748
[20]
Doupa, D., Boye, O., Ndiaye, M., Djité, M., Matar-Kandji, P., Seck, M., et al. (2018) Prevalence of Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency (A-376/202) in Homozygous Sickle Cell Patients in Senegal and Its Clinical Impact. African Journal of Biochemistry Research, 12, 55-62. https://doi.org/10.5897/AJBR2018.0988
[21]
Simpore, J., Nitiema, H., Ouedraogo, P., Sawadogo, L., Ilboudo, S., Maria, E., et al. (2007) Glucose-6-Phosphate Déshydrogénase and Sickle Cell Disease in Burkina-Faso. Pakistan Journal of Biological Sciences, 10, 409-414. https://doi.org/10.3923/pjbs.2007.409.414
[22]
Kambale-Kombi, P., Marini, R., Atoba Bokelea, C., Bours, V., Kayembe, C., Batina-Agasa, S., et al. (2022) Does Glucose-6-Phosphate Dehydrogenase Deficiency Worsen the Clinical Features of Sickle Cell Disease? A Multi-Hospital-Based Cross-Sectional Study. Hematology, 27, 590-595. https://doi.org/10.1080/16078454.2022.2074715
