بررسي تاثير هيدروكسي اوره بر ميزان توليد هموگلوبين جنيني و فراواني حملات دردناك در كم خوني سلول داسي شكل

EFFECT OF HYDROXYUREA ON FETAL HEMOGLOBIN PRODUCTION AND FREQUENCY OF PAINFUL CRISES IN SICKLE CELL ANEMIA

Hydroxyurea (HU) enhances the production of fetal hemoglobin (HbF) and on the other hand this event can decreases the frequency of painful crises in sickle cell anemia (SCA). We studied the efficiency of HU in reducing painful crises and itʹs effect on HbF production rate and on other hematologic values in 40 SCA patients. Median age of patients was 20 years. Eighteen were male and 22 were female. Duration of study was 2 years. In the first year, patients were under observation without HU but were on folic acid I mgld and necessary data collected, then HU started at a dosage of 20-25 mg/kg/day in adition to folic acid and continued for one year. Among treated patients with HU,HbF level increased significantly in 70% of patients (mean 4% vs 10.8%, p<0.001). Changes in the hemoglobin level and platelers numbers were not significant as HbF but leukocytes and reticulocytes number decreased significantly. Patients treated with HU had lower annual rates of painful crises in comparison to first year of study without HU therapy (mean 3 vs 5.7 crises per year, p<0.001) and crises interval increased significantly(p<0.001). Treatment with above doses of HU had not any important adverse effect. We concluded that treatment with HU can increase HbF production rate and subsequently improves the clinical course of SCA.