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Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia

Perla Vicari1, Adriana Barretto de Mello, Maria Stella Figueiredo

  • 1Disciplina de Hematologia e Hemoterapia, Universidade Federal de São Paulo-EPM, São Paulo, Brazil. perlavicari@hotmail.com

American Journal of Hematology
|February 24, 2005
PubMed

Fetal hemoglobin (HbF) inhibits the polymerization of sickle hemoglobin, modulating the clinical features of sickle cell anemia (SCA). Hydroxyurea (HU) therapy can increase the HbF level, although its production can be influenced by genetic determinants. Twenty-two Brazilian SCA patients were evaluated over 5 years before and after HU use. We analyzed (1) betaS haplotype; (2) patient characteristics; and (3) toxicity. No differences between age, sex, and HU response were observed. We found 40.9% of homozygous for Bantu haplotype, and, in contrasting to other trials, we observed HbF level increase in this group (3.84-9.08 g/dL, P=0.003). Adverse effects were rare. Labyrinthitis was observed in 2 (9.10%) patients after HU use, although this complication had not been described before.

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