Sickle cell anemia is a heterogeneous disorder with variable severity. Initial observations showed that a high level of fetal hemoglobin (HbF) was associated with minor clinical manifestations, as HbF interferes with HbS polymerization. Hydroxyurea, a cytotoxic agent, has several effects on sickle cell patients, such as: increased HbF production, increased mean corpuscular volume, improved red blood cell hydration, improved hemoglobin, myelosuppression, production of nitric oxide and decreases in adhesion molecules. Till now, hydroxyurea is considered the most successful drug therapy for sickle cell anemia and its use is described in this article.
Sickle cell anemia; hydroxyurea; HbF synthesis; treatment