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Erythroid progenitors in the peripheral blood of children with sickle cell disease
T B West1, K Ohene-Frempong, C J Stoeckert
1Department of Pediatrics, Children's Hospital of Philadelphia, PA 19104.
Insights
Pediatric sickle cell disease (SCD) patients show higher burst forming units-erythroid (BFU-E) counts than healthy children. BFU-E numbers strongly decrease with age in these children.
Area of Science:
- Hematology
- Pediatric Medicine
- Sickle Cell Disease Research
Background:
- Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin.
- Erythropoiesis, the production of red blood cells, is often dysregulated in SCD.
- Burst forming units-erythroid (BFU-E) are early erythroid progenitor cells crucial for red blood cell development.
Purpose of the Study:
- To quantify peripheral blood burst forming units-erythroid (BFU-E) in pediatric sickle cell disease (SCD) patients.
- To investigate the correlation between circulating BFU-E levels and fetal hemoglobin (HbF) in children with SCD.
- To determine the relationship between BFU-E count and age in pediatric SCD.
Main Methods:
- Peripheral blood samples were collected from pediatric SCD patients (under 18) in a stable disease state.
- Fetal hemoglobin (HbF) levels and BFU-E numbers were quantified in SCD patients.
- Data from SCD patients were compared to those from age-matched healthy children.
Main Results:
- Children with SCD exhibited significantly higher peripheral blood BFU-E counts compared to normal controls (30.7 vs. 15.7 per 10^5 mononuclear cells; p=0.009).
- A trend towards a direct relationship between HbF levels and BFU-E number was observed (p=0.06).
- A strong inverse correlation was found between BFU-E number and age in pediatric SCD patients (p<0.0001).
Conclusions:
- Pediatric SCD patients have an elevated number of peripheral blood BFU-E compared to healthy children.
- Unlike adults, children with SCD do not show an inverse relationship between HbF levels and BFU-E counts.
- The number of BFU-E in pediatric SCD patients significantly decreases with increasing age, suggesting a role in erythropoietic stress.
Purpose:
The goals of this study were (a) to determine the number of peripheral blood burst forming units-erythroid (BFU-E); (b) to define the relationship between circulating BFU-E number and fetal hemoglobin (HbF) level; and (c) to define the relationship between BFU-E number and age in pediatric sickle cell disease (SCD) patients.
Patients And Methods:
Fetal hemoglobin (HbF) level and peripheral blood BFU-E number were determined in children < 18 years of age with SCD in a steady state of their disease. These data were compared with those of normal children.
Results:
An increased number of BFU-E was observed in the peripheral blood of children with SCD compared with normals (30.7 vs. 15.7 per 10(5) mononuclear cells, respectively; p = 0.009). Overall there was the suggestion of a direct relationship between HbF level and peripheral blood BFU-E number (regression coefficient = 0.445; p = 0.06). Additionally, a strong inverse relationship between BFU-E number and age (regression coefficient = -0.671; p < 0.0001) was observed.
Conclusions:
In children with SCD (a) there are an increased number of peripheral blood BFU-E compared with normal children; (b) the inverse relationship between HbF level and BFU-E number observed in adult SCD patients is not seen in children; and (c) there is a strong inverse relationship between age and BFU-E number. This information may help to further clarify the relationship between peripheral blood BFU-E and erythropoietic stress.