Publication:

Impact of hydroxyurea use on severity and hematologic profile in children with sickle cell disease at University of Benin Teaching Hospital, Benin city, Nigeria

Background Sickle cell disease (SCD) is a major cause of morbidity and mortality among children in sub-Saharan African. Hydroxyurea (HU) remains the cornerstone of disease- modifying therapy, but its utilization, hematologic impact and relationship with disease severity among Nigerian children are not fully characterized. This study assessed the impact of hydroxyurea use on disease severity and haematologic profile in children with SCD attending the University of Benin of teaching Hospital, Benin city.

Methods This cross- sectional study included 225 children with SCA aged 1-18 years in steady state. Data were obtained on socio- demographic characteristics, HU use and disease severity were using structured proforma and medical records. Disease severity was assessed using Adegoke’s scoring system while haematologic parameters including Packed cell volume and White blood cell counts were documented. Statistical associations between HU, hematologic indices and disease severity were analysed using Chi-square tests, with significance set at p<0.05.

Results The mean age was 9.5±4.5 years: 57.8% were male and 57.3% belonged to the middle socioeconomic class. Most patients had mild (51.1%) or moderate (47.6%) disease with severe disease in only 1.3%. disease severity was significantly associated with gender (p=0.029). Hydroxyurea use was high (61.3%), more frequent in females (p=0.032). HU use was significantly associated with disease severity (ꭓ2=9.422; p=0.009). Higher PCV was linked with milder disease (p= 0.004) whereas elevated WBC counts were associated with more severe disease (p<0.001).

Conclusions HU therapy, higher PCV and lower WBC counts are associated with milder disease among children with SCD. These findings reinforce Hydroxyurea clinical benefits and highlight the value of simple haematologic indices for disease monitoring in resource poor settings.