AN ANALYSIS OF CLINICAL AND LABORATORY FINDINGS OF HEMOGLOBINOPATHIES AND THEIR DISTRIBUTION IN KHOZESTAN PROVIENCE

In Iran, sickle cell disease (SCD) is often mild with a high level of hemoglobin. Furthermore, there is lack of sufficient published research works on the nature, prevalence, clinical features and distinct mutations involved in SCD in Khozestan, a province in the southwest of Iran. Serial evaluation of patients and their family using hemoglobin electrophoresis, CBC, RBC indices and clinical feature conducted on 28 SCD and 139 other concurrent associated thalassemias or sickle trait over 8 years of follow-up, showed SCD to be the second most common congenital inherited hemoglobinopathy disorder second to -thalassemia syndrome among the referral cases to Ahwaz Thalassemia and Hemoglobinopathy Research Center. The pattern of geographical distribution showed that the number of patients was on an creasing trend from the south and southwest in toward central part of this province. This area is where the Arab population are mainly living. The family screening also showed that the 97% of carrier states are among the ethnic Arab tribes of Khvozestan, from which only 10% were Arab-Persian half breeds and only 3% were non-Bakhtiry Persians. No Bakhtiary origin was found among the SCD patients referred to our research center during the 8-year period of this study. These findings showed that the SCD in the Khozestan to be different from that of black Africans. Their general appearance is usually good-looking Caucasian boys and girls.