Evaluation of hematological variables according to haplotypes in patients with users and nonusers of hydroxyurea in the sickle cell anemia

Clinical and prognostic profile of patient with sickle cell disease may vary according to their type of haplotype and fetal hemoglobin level. Objective: Relate hematological variables to the haplotypes of patients with sickle cell anemia and whether or not they use hydroxyurea. Material and methods: Cross-sectional study of patients with sickle cell anemia users and non-users of hydroxyurea. The hematological analyzes were submitted to laboratory tests as erythrogram and reticulocyte count. The dosage of HbF was obtained by means of High-performance liquid chromatography. Deoxyribonucleic acid was extracted by the phenol/chloroform method for confirmation of HbS and identification of haplotypes by PCR/RFLP. Results: Laboratory parameters according to genotypes presented a statistically significant difference for HbF (p=0.008). The mean Hb concentration (8.9±1.1 g/dL), hematocrit (26.1±3.1%), HCM (34.4±5.0 pg) and VCM (99.9±13.5 fL) were statistically significant (p<0.001). The mean HbF level (15.6±7.4%) presented a statistically significant difference (p=0.001) in subjects taking hydroxyurea. The CAR/CAR and CAR/Ben genotypes showed high levels of HbF. Conclusion: Indication of the use of hydroxyurea predominates in patients with at least one CAR chromosome. Laboratory parameters Hb,VCM, HCM and HbF show better results in individuals using hydroxyurea. The CAR/CAR and CAR/Ben genotype gives higher levels of HbF.