Circulating BFU-E in sickle cell anemia: relationship to percent fetal hemoglobin and BPA-like activity.
Circulating 14-day erythroid progenitors (BFU-E) from sickle cell anemia (SS) patients were studied in culture to determine their frequency and their sensitivity to erythropoietin (Epo). Increased numbers of circulating BFU-E were found in half of the patients studied, whereas the remainder had a normal count. Patients with high circulating BFU-E counts had lower fetal hemoglobin (HbF) percentages (congruent to 4.5%) than patients with low circulating BFU-E counts (HbF congruent to 13%). This difference was highly significant (p less than 0.0001). In addition, SS circulating BFU-E expressed increased sensitivity to Epo due, at least partially, to an increased production of burst-promoting activity-like factor(s) generated by light-density mononuclear cells. These findings emphasize the possible role of the HbF level (and the extent of sickling) in BFU-E regulation under the continuous hemopoietic stress of SS disease.
Croizat
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Division of Hematology, Albert Einstein College of Medicine, Bronx, New York 10461.
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D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood*; D003114:Colony-Forming Units Assay; D004305:Dose-Response Relationship, Drug; D004903:Erythrocyte Aggregation; D004912:Erythrocytes / Q000166:cytology* / Q000187:drug effects; D004921:Erythropoietin / Q000494:pharmacology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D013997:Time Factors
D004921:Erythropoietin