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Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea. Multicenter Study of Hydroxyurea.
Blood 1997; 89(3):1078-88Blood

Abstract

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.

Authors+Show Affiliations

Veterans Affairs Medical Center, Jackson, MS 39216, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Clinical Trial
Journal Article
Multicenter Study
Randomized Controlled Trial
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, Non-P.H.S.
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

9028341

Citation

Steinberg, M H., et al. "Fetal Hemoglobin in Sickle Cell Anemia: Determinants of Response to Hydroxyurea. Multicenter Study of Hydroxyurea." Blood, vol. 89, no. 3, 1997, pp. 1078-88.
Steinberg MH, Lu ZH, Barton FB, et al. Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea. Multicenter Study of Hydroxyurea. Blood. 1997;89(3):1078-88.
Steinberg, M. H., Lu, Z. H., Barton, F. B., Terrin, M. L., Charache, S., & Dover, G. J. (1997). Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea. Multicenter Study of Hydroxyurea. Blood, 89(3), pp. 1078-88.
Steinberg MH, et al. Fetal Hemoglobin in Sickle Cell Anemia: Determinants of Response to Hydroxyurea. Multicenter Study of Hydroxyurea. Blood. 1997 Feb 1;89(3):1078-88. PubMed PMID: 9028341.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea. Multicenter Study of Hydroxyurea. AU - Steinberg,M H, AU - Lu,Z H, AU - Barton,F B, AU - Terrin,M L, AU - Charache,S, AU - Dover,G J, PY - 1997/2/1/pubmed PY - 1997/2/1/medline PY - 1997/2/1/entrez SP - 1078 EP - 88 JF - Blood JO - Blood VL - 89 IS - 3 N2 - 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. SN - 0006-4971 UR - https://www.unboundmedicine.com/medline/citation/9028341/Fetal_hemoglobin_in_sickle_cell_anemia:_determinants_of_response_to_hydroxyurea__Multicenter_Study_of_Hydroxyurea_ L2 - http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=9028341 DB - PRIME DP - Unbound Medicine ER -