Abstract
Background: Sickle cell anemia (SCA) is a major global public health concern. Fetal hemoglobin level (HbF) is a major modulating factor of the clinical manifestation of the disease. In Nigeria, data on HbF level of children with SCA and its relationship with the spleen, a major reticuloendothelial organ, are scanty. Methods: In this cross-sectional comparative study, we determined steady-state HbF levels of children with SCA aged 1–15 years by high-performance liquid chromatography and compared with their age-, sex-, and socioeconomic class-matched HbAA controls. The spleen size was determined clinically and sonographically. Relationships between HbF level and spleen size and malaria parasite density were also determined. Results: About two-thirds of children with SCA had HbF level of 0.05. Conclusion: Children with SCA had higher HbF levels compared with matched controls. Furthermore, patients with autosplenectomy had significantly lower HbF levels. Further studies exploring the roles of foetal haemoglobin on spleen functions in children with SCA are advocated, since spleen dysfunction is central to morbidity and mortality in this group of patients.
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