Haemoglobin F and A2 in Nigerian children with sickle cell anaemia

Area of Science:

• Hematology
• Genetics
• Pediatrics

Background:

• Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin.
• Hemoglobin F (HbF) and Hemoglobin A2 (HbA2) are types of hemoglobin that can influence SCD severity.
• Understanding HbF and HbA2 levels in pediatric SCD patients is crucial for disease management.

Purpose of the Study:

• To determine and compare the concentrations of HbF and HbA2 in children with sickle cell disease (Hb SS) versus healthy controls.
• To investigate whether HbF levels fluctuate between stable states and vaso-occlusive crises in pediatric Hb SS patients.

Main Methods:

• Quantification of hemoglobin F and A2 concentrations using established laboratory techniques.
• Comparison of HbF and HbA2 levels between a cohort of 132 children with Hb SS (ages 6 months to 16 years) and 50 age- and sex-matched controls.
• Analysis of HbF levels in 18 Hb SS patients during both stable periods and vaso-occlusive crises.

Main Results:

• Mean HbF concentration was significantly higher in Hb SS patients (9.5%) compared to controls (1.5%).
• Mean HbA2 concentrations showed minimal difference between Hb SS patients (2.7%) and controls (2.4%).
• No significant difference in HbF concentration was observed between stable and crisis states in Hb SS patients.

Conclusions:

• Children with sickle cell disease (Hb SS) exhibit elevated levels of fetal hemoglobin (HbF).
• Hemoglobin A2 (HbA2) levels are comparable between pediatric Hb SS patients and controls.
• Fetal hemoglobin (HbF) levels remain stable regardless of the clinical state (stable vs. crisis) in pediatric sickle cell disease.