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

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.