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Fetal hemoglobin in sickle cell anemia: determinants of response to
hydroxyurea. Multicenter Study of Hydroxyurea
MH Steinberg, ZH Lu, FB Barton, ML Terrin, S Charache and GJ Dover
Veterans Affairs Medical Center, Jackson, MS 39216, USA.
Hydroxyurea (HU) can increase fetal hemoglobin (HbF) in sickle cell anemia
(HbSS). To identify determinants of the HbF response, we studied 150
HU-treated patients grouped by quartiles of change in HbF from baseline to
2 years. Half of the HU-assigned patients had long-term increments in HbF.
In the top two quartiles, HbF increased to 18.1% and 8.8%. These patients
had the highest baseline neutrophil and reticulocyte counts, and largest
treatment-associated decrements in these counts. In the lower two
quartiles, 2-year HbF levels (4.2% and 3.9%) and blood counts changed
little from baseline. In the highest HbF response quartile,
myelosuppression developed in less than 6 months, compliance was best, and
final doses of HU were 15 to 22.5 mg/kg. All four quartiles had substantial
increases of F cells in the first year. This was maintained for 2 years
only in the top three quartiles. Leukocyte and reticulocyte counts
decreased initially in all quartiles, but drifted back toward baseline
levels in the lowest HbF response quartile. Initial HbF level and phenotype
of the F-cell production (FCP) locus were not associated with HbF response,
but absence of a Central African Republic (CAR) haplotype was. Bone marrow
ability to withstand HU treatment may be important for sustained HbF
increases during HU treatment of HbSS.
Volume 89,
Issue 3,
pp. 1078-1088,
02/01/1997
Copyright © 1997 by The American Society of Hematology
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