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Fetal Hemoglobin and F-Cell Responses to Long-Term Hydroxyurea
Treatment in Young Sickle Cell Patients
Micheline Maier-Redelsperger,
Mariane de Montalembert,
Antoine Flahault,
Maria Grazia Neonato,
Rolande Ducrocq,
Marie-Pierre Masson,
Robert Girot, and
Jacques Elion for the French Study Group
on Sickle Cell Disease
From the Service d'Hématologie Biologique, Hôpital
Tenon, Paris, France; Unité INSERM U 458, Hôpital Robert
Debré, Paris, France; the Centre de Transfusion Sanguine,
Hôpital Necker-Enfants Malades, Paris, France; and Unité
INSERM 444, Hôpital Saint Antoine, Paris, France.
We have studied the cellular and molecular responses to long-term
hydroxyurea (HU) treatment in 29 severely affected young patients with
sickle cell disease (mean age, 10.9 ± 4.1 years). Patients received
HU at 20 mg/kg/d on 4 consecutive days per week initially, with a
monthly escalated dose avoiding marrow-toxicity (mean steady-state
dose, 34.2 ± 4.6 mg/kg/d) for 12 to 36 months (mean duration, 22 months). The studied parameters were hemoglobin F (HbF), F
reticulocytes (F retics), F cells, the amount of HbF per F cell (F/F
cell), polymer tendency at 40% and 70% oxygen saturation, and
hemolysis. Initial HbF (Fi) was dispersed (from 0.85% to 13.9%). HbF
increased in all patients but 1. HbF at maximal response (Fmax) reached
a sustained level varying from a 1.5-fold to a 16-fold Fi after a
variable delay (6 to 24 months). Fmax was not related to HU dosage, but
 F (Fmax  Fi) was strongly correlated to MCV (MCVmax MCVi).
HbF increase resulted from the increase of both F cells and F/F cell.
In this rather short series, Fi and Fmax were not significantly
associated with age, gender, or  -globin haplotype. Neither Fmax nor
F was related to bone marrow reserve, as measured by baseline
reticulocyte or neutrophil counts. However, Fmax was highly dependent
on Fi. When patients are individualized into three groups according to
Fmax (group 1, Fmax >20% [12 patients]; group 2, 10% < Fmax < 20% [11 patients]; group 3, Fmax <10% [5 patients]), Fi is
significantly different between groups, being the highest in group 1. In addition, the best responders (group 1) were significantly different
from patients in the two other groups with higher levels of total
hemoglobin, decreased bilirubin, and decreased polymer tendency.
Blood, Vol. 91 No. 12 (June 15), 1998:
pp. 4472-4479
© 1998 by The American Society of Hematology.
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