Heterogeneity in the properties of burst-forming units of erythroid lineage
in sickle cell anemia: DNA synthesis and burst-promoting activity
production is related to peripheral hemoglobin F levels
H Croizat, HH Billett and RL Nagel
Department of Medicine, Albert Einstein College of Medicine/Montefiore
Medical Center, Bronx, NY.
Circulating 14-day erythroid progenitors (BFU-E) from 28 sickle cell anemia
(SS) patients with hemoglobin F (HbF) levels ranging from 2% to 16% were
studied to determine their sensitivity to [3H] thymidine kill and
burst-promoting activity (BPA)-like factor production. We find that the
proportion of BFU-E sensitive to 3H-dT kill, and hence active in DNA
synthesis, was inversely correlated with the percent of peripheral HbF when
light density (LD) mononuclear cells were used for plating. Regression
analysis showed that the correlation between HbF level and BFU-E kill was
highly significant (r = .88; P less than .00003). We confirmed the BPA-like
factor(s) production by LD mononuclear cells of SS patients, and found, in
addition, that this phenomenon is restricted to the population of SS
patients with HbF levels lower than 9%. Circulating BFU-E of patients with
high HbF levels are not sensitive to 3H-dT, and their mononuclear cells do
not release BPA-like factor. In summary, SS patients exhibit differences in
the capacity of their mononuclear cells to produce BPA activity according
to their peripheral HbF level, as well as to the DNA synthesis-state of
their circulating BFU-E. We conclude that erythroid progenitors differ
among SS patients in relation to their peripheral HbF level.
Volume 75,
Issue 4,
pp. 1006-1010,
02/15/1990
Copyright © 1990 by The American Society of Hematology
