The Influence of Fetal Hemoglobin on Hematological Parameters and Clinical Severity in Patients with Homozygous Sickle Cell Disease.

Abstract

Background: In Sickle cell disease (SCD), fetal hemoglobin (HbF) influences clinical severity, resulting in variable disease phenotypes. This study evaluated HbF levels, hematological parameters, and clinical severity in homozygous SCD patients.

Methodology: A cross-sectional observational study with the prospective recruitment of 40 patients with homozygous SCD was conducted. A standardized questionnaire collected demographic data. Clinical and laboratory assessments calculated the clinical severity scores. Analyses used Stata/MP version 17.0; significance was set at p < 0.05.

Results: HbF levels were inversely correlated with age (ρ = -0.998) and clinical severity (ρ = -0.928) but positively correlated with Hb (ρ = 0.999), RBC count (ρ = 0.999), and Packed Cell Volume (PCV) (ρ = 0.965) (all p < 0.001). Clinical severity was positively associated with age (ρ = 0.929) but negatively associated with Hb (ρ = -0.930), RBC count (ρ = -0.930), and PCV (ρ = -0.916) (all p < 0.001). HbF was the only independent predictor of disease severity (β = -0.14, 95% CI: -0.22 to -0.05, p = 0.002).

Conclusion: Increased HbF levels were associated with improved hematological parameters and reduced clinical manifestations and clinical severity in homozygous SCD patients, supporting the role of HbF as a biomarker for disease severity.