Erythroid progenitors in the peripheral blood of children with sickle cell disease.
OBJECTIVE
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.


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.
West
T B
TB
Department of Pediatrics, Children's Hospital of Philadelphia, PA 19104.
Ohene-Frempong
K
K
Stoeckert
C J
CJ
Surrey
S
S
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D004906:Erythrocyte Count; D015672:Erythroid Precursor Cells; D005319:Fetal Hemoglobin / Q000032:analysis; D006801:Humans
D005319:Fetal Hemoglobin