Abstract:
This is prospective, analytical and cross- sectional study carried out in Medical Army hospital in Khartoum State, during April 2015 - July 2015.
Forty sickle cell disease patients were studied, 18 were males and 22 were females, their ages 1-13 years, 23(58%) were sickle cell anemia (HbSS), 17(42%) were sickle cell trait (HbAS)as known cases by hemoglobin electrophoresis.
The aim of this study to measure the level different hemoglobin types in the study group and comparison of this hemoglobin types inboth homozygote and heterozygote states of sickle cell disease patients.
Estimation hemoglobin types were carried out by CAPILLARYS 2 FLEX-PIERCING instrument and the results were analyzed by SPSS computer programme.
The result revealed that the level of HbF in sickle cell disease patients is (7.2 ± 4.9) where as the level of HbA ,HbA2 and HbS were )78.5 ± 19.2), (3.1± 0.64) and (11.2 ± 21.5).
The level of HbS is the higher indicate that most patients have large level of HbS which is the diagnostic hemoglobin for sickle cell anemia.
The mean of HbF is increased because all patients given hydroxyurea and other supportive treatment. HbA2 show normal value, while HbA is decreased.
HbF, HbS, HbA2 and HbA (%) show different means and std Deviations in males and females as HbF % in males (6.3 ± 3.5) while in females (8.1± 5.7), HbS % in males (75.4 ± 24.6) while in females (80.9 ± 13.3), HbA2 (%) in males (3.3 ± 0.66) while in females (2.9 ± 0.60) and HbA (%) in males (14.9 ± 27.5) while in females (8.2 ± 15.0).
The mean andstandard deviation of different hemoglobin types in homozygous patients (SS) andheterozygous patients (AS) are HbF (%) in (HbSS) patients (9.7 ± 4.5) while in (HbAS) patients (3.9 ± 3.3).
Fetal Hb is higher in homozygous than heterozygous, it is concluded that sickle cell anemia patients HbF level more than sickle cell trait patients.
HbS (%) in (HbSS) patients (87.1 ± 4.3) while in (HbAS) patients (66.7 ± 24.8) there is adifference in HbS in homozygous and heterozygous patients, sickler Hb is higher in homozygous than heterozygous and this is because homozygous have two copies of HbS while heterozygous have just one copy of HbS.
HbA2 (%) in (HbSS) patients (3.3 ± 0.66) while in (HbAS) patients (2.9 ± 0.56), means no difference in HbA2 in homozygous and heterozygous states.
HbA (%) in (HbSS) patients (.000 ± .000) while in (HbAS) patients (26.4 ± 26.5), adult Hb is higher in heterozygous than homozygous and this is because homozygous have no HbA gene while heterozygous have one copy of HbA gene.
Correlation between HbF (%) and age (years) among the study group show
Strong negative correlation.
Correlation between HbS (%) and age (years) among the study group show
Strong negative correlation.
Correlation between HbA2 (%) and age (years) among the study group show
Weak negative correlation.
Correlation between HbA (%) and age (years) among the study group show
positive correlation.
