TITLE:
Influence of Hemoglobin S Haplotypes on the Responses to Hydroxyurea Treatment in Children with Sickle Cell Disease in Abidjan, Côte d’Ivoire
AUTHORS:
Mireille Aye-Yayo, Vincent Yapo, Boidy Kouakou, Missa Louis Adjé, Adia Eusèbe Adjambri, Ebah Hermance Kassi, Taïratou Kamagate, Duni Sawadogo
KEYWORDS:
Sickle Cell Disease, Children, Haplotype, Hydroxyurea, Côte d’Ivoire
JOURNAL NAME:
Open Journal of Genetics,
Vol.14 No.1,
March
21,
2024
ABSTRACT: Background: In Côte d’Ivoire so
far, the circulating haplotypes have been inferred on the phenotypic profiling
of SCD patients. The impact of the circulating haplotypes on the use of
Hydroxyurea has not been assessed yet. Therefore the objective of this study is
to identify in Abidjan the HbS haplotypes that modulate HU treatment responses. Methods: In a cross-sectional descriptive and analytical study, children
aged 5 to 15 years with SCD, and carrying the hemoglobin phenotypes SSFA2 and
SFA2, were recruited into a HU treatment cohort. Various parameters on the
haplotypes and the outcomes of the treatment were analyzed. Results: Thirty nine children with SCD were included. The phenotypic profile of the cohort
was 86.6% of SSFA2 and 15.4% of SFA2. Three haplotypes were found, the Benin
haplotype, the Senegal haplotype, and an atypical one. The participants
belonged to three genotypes, Benin/atypical
(64.1%), Benin/Senegal (33.3%) and Senegal/Senegal (2.6%). Overall, HU
treatment was successful in all haplotypes with 12 out of 39 patients failing
treatment after 12 months in the Benin haplotype group. The association between
HU treatment success and the Benin haplotype was found in terms of the decrease
in the number of white blood cells and the students missing class. Conclusion: The study revealed that inferring haplotype based on the phenotypic profile
could be inaccurate. The proportion of atypical haplotype that were not
previously described in Côte d’Ivoire was high. All the haplotypes seemed to be associated with HU
treatment success but some patients with Benin haplotype did not respond well.